Pigmentation Disorders

The Diagnosis: Blaschkitis

A punch biopsy from the right lateral hip was performed. Histopathologic examination revealed orthokeratosis overlying mild psoriasiform epidermal hyperplasia associated with a lichenoid infiltrate composed almost entirely of lymphocytes (Figure). The infiltrate did not entirely obscure the dermoepidermal junction and spared the adnexal structures. The clinical presentation along with histopathologic analysis confirmed a diagnosis of blaschkitis. The lesions were treated with triamcinolone ointment twice daily as needed, and the patient reported symptomatic and clinical improvement with this intervention at 4-week follow-up.

Described by Grosshans and Marot¹ in 1990, blaschkitis is an acquired inflammatory dermatosis following the lines of Blaschko. It predominantly is seen on the trunk and typically presents with pruritic papules and vesicles. It frequently has a relapsing course and is more commonly found in adults. Blaschkitis is considered a form of cutaneous mosaicism representing somatic mutations affecting epidermal cell growth and migration during embryogenesis. It has been proposed that these aberrant cells are not clinically apparent at birth; however, viral infection and drug or other environmental triggers can induce antigen presentation of the clone cells activating a T cell–mediated inflammatory response.^2-4

The differential diagnosis includes other acquired Blaschko-linear dermatoses such as lichen striatus, inflammatory linear verrucous epidermal nevus, unilateral lichen planus, linear lichen sclerosus, linear psoriasis, linear fixed drug reaction, lichen nitidus, and others.^1,4 Given the overlap in clinical and histopathological presentations of the entities in the differential, it often is difficult to discern the etiology of the papular and vesicular eruption in question. Discrimination of one etiology from the others is further confounded by the fact that these lesions can all be pruritic and initially are treated with topical corticosteroids. A degree of clinical suspicion for blaschkitis coupled with prior understanding of lesional manifestations is helpful in this circumstance. Although classic lichen planus often affects the arms, legs, flexor surfaces, and occasionally the oral mucosa, blaschkitis generally is limited to the trunk. The lesions of lichen planus generally are violaceous, flattopped, polygonal papules that tend to coalesce. They often have a thin, transparent, and adherent scale overlying the papular lesions, and they occasionally demonstrate Wickham striae, which are faint white reticulated networks typically seen in oral mucosal lesions. In the case of lichen nitidus, lesions often follow a geometric line due to the Köbner response, whereas physical trauma from scratching or injury causes lesions to form along the line of insult. Assessing patients for any newly initiated medications can help eliminate lichenoid drug eruptions. Lichen striatus perhaps has the most overlap with blaschkitis, having been described as also following the lines of Blaschko but occurring in children rather than adults. Inflammatory linear verrucous epidermal nevi also can be distinguished from blaschkitis on this premise, as these lesions arise during the first 5 years of life and generally affect the lower extremities.^4,5

Histopathology is somewhat variable but generally includes spongiotic dermatitis with concomitant interface
dermatitis characterized by T-cell infiltration. Spongiosis is a feature less commonly seen in lichen striatus. Lesions can progress over time from spongiotic dermatitis to spongiotic psoriasiform dermatitis and later to spongiotic psoriasiform lichenoid dermatitis.⁴ Treatment of blaschkitis should begin with reassurance of the benign nature of the dermatosis. Pruritic symptoms can be managed with a course of topical steroids.

Blaschkitis is a rare and self-limiting acquired dermatosis that should be incorporated into the differential diagnosis of Blaschko-linear dermatoses. Further investigation is needed to determine if blaschkitis and lichen striatus represent the ends of a disease spectrum or completely distinct entities.

The Diagnosis: Blaschkitis

A punch biopsy from the right lateral hip was performed. Histopathologic examination revealed orthokeratosis overlying mild psoriasiform epidermal hyperplasia associated with a lichenoid infiltrate composed almost entirely of lymphocytes (Figure). The infiltrate did not entirely obscure the dermoepidermal junction and spared the adnexal structures. The clinical presentation along with histopathologic analysis confirmed a diagnosis of blaschkitis. The lesions were treated with triamcinolone ointment twice daily as needed, and the patient reported symptomatic and clinical improvement with this intervention at 4-week follow-up.

Described by Grosshans and Marot¹ in 1990, blaschkitis is an acquired inflammatory dermatosis following the lines of Blaschko. It predominantly is seen on the trunk and typically presents with pruritic papules and vesicles. It frequently has a relapsing course and is more commonly found in adults. Blaschkitis is considered a form of cutaneous mosaicism representing somatic mutations affecting epidermal cell growth and migration during embryogenesis. It has been proposed that these aberrant cells are not clinically apparent at birth; however, viral infection and drug or other environmental triggers can induce antigen presentation of the clone cells activating a T cell–mediated inflammatory response.^2-4

The differential diagnosis includes other acquired Blaschko-linear dermatoses such as lichen striatus, inflammatory linear verrucous epidermal nevus, unilateral lichen planus, linear lichen sclerosus, linear psoriasis, linear fixed drug reaction, lichen nitidus, and others.^1,4 Given the overlap in clinical and histopathological presentations of the entities in the differential, it often is difficult to discern the etiology of the papular and vesicular eruption in question. Discrimination of one etiology from the others is further confounded by the fact that these lesions can all be pruritic and initially are treated with topical corticosteroids. A degree of clinical suspicion for blaschkitis coupled with prior understanding of lesional manifestations is helpful in this circumstance. Although classic lichen planus often affects the arms, legs, flexor surfaces, and occasionally the oral mucosa, blaschkitis generally is limited to the trunk. The lesions of lichen planus generally are violaceous, flattopped, polygonal papules that tend to coalesce. They often have a thin, transparent, and adherent scale overlying the papular lesions, and they occasionally demonstrate Wickham striae, which are faint white reticulated networks typically seen in oral mucosal lesions. In the case of lichen nitidus, lesions often follow a geometric line due to the Köbner response, whereas physical trauma from scratching or injury causes lesions to form along the line of insult. Assessing patients for any newly initiated medications can help eliminate lichenoid drug eruptions. Lichen striatus perhaps has the most overlap with blaschkitis, having been described as also following the lines of Blaschko but occurring in children rather than adults. Inflammatory linear verrucous epidermal nevi also can be distinguished from blaschkitis on this premise, as these lesions arise during the first 5 years of life and generally affect the lower extremities.^4,5

Histopathology is somewhat variable but generally includes spongiotic dermatitis with concomitant interface
dermatitis characterized by T-cell infiltration. Spongiosis is a feature less commonly seen in lichen striatus. Lesions can progress over time from spongiotic dermatitis to spongiotic psoriasiform dermatitis and later to spongiotic psoriasiform lichenoid dermatitis.⁴ Treatment of blaschkitis should begin with reassurance of the benign nature of the dermatosis. Pruritic symptoms can be managed with a course of topical steroids.

Blaschkitis is a rare and self-limiting acquired dermatosis that should be incorporated into the differential diagnosis of Blaschko-linear dermatoses. Further investigation is needed to determine if blaschkitis and lichen striatus represent the ends of a disease spectrum or completely distinct entities.

The Diagnosis: Blaschkitis

A punch biopsy from the right lateral hip was performed. Histopathologic examination revealed orthokeratosis overlying mild psoriasiform epidermal hyperplasia associated with a lichenoid infiltrate composed almost entirely of lymphocytes (Figure). The infiltrate did not entirely obscure the dermoepidermal junction and spared the adnexal structures. The clinical presentation along with histopathologic analysis confirmed a diagnosis of blaschkitis. The lesions were treated with triamcinolone ointment twice daily as needed, and the patient reported symptomatic and clinical improvement with this intervention at 4-week follow-up.

Described by Grosshans and Marot¹ in 1990, blaschkitis is an acquired inflammatory dermatosis following the lines of Blaschko. It predominantly is seen on the trunk and typically presents with pruritic papules and vesicles. It frequently has a relapsing course and is more commonly found in adults. Blaschkitis is considered a form of cutaneous mosaicism representing somatic mutations affecting epidermal cell growth and migration during embryogenesis. It has been proposed that these aberrant cells are not clinically apparent at birth; however, viral infection and drug or other environmental triggers can induce antigen presentation of the clone cells activating a T cell–mediated inflammatory response.^2-4

The differential diagnosis includes other acquired Blaschko-linear dermatoses such as lichen striatus, inflammatory linear verrucous epidermal nevus, unilateral lichen planus, linear lichen sclerosus, linear psoriasis, linear fixed drug reaction, lichen nitidus, and others.^1,4 Given the overlap in clinical and histopathological presentations of the entities in the differential, it often is difficult to discern the etiology of the papular and vesicular eruption in question. Discrimination of one etiology from the others is further confounded by the fact that these lesions can all be pruritic and initially are treated with topical corticosteroids. A degree of clinical suspicion for blaschkitis coupled with prior understanding of lesional manifestations is helpful in this circumstance. Although classic lichen planus often affects the arms, legs, flexor surfaces, and occasionally the oral mucosa, blaschkitis generally is limited to the trunk. The lesions of lichen planus generally are violaceous, flattopped, polygonal papules that tend to coalesce. They often have a thin, transparent, and adherent scale overlying the papular lesions, and they occasionally demonstrate Wickham striae, which are faint white reticulated networks typically seen in oral mucosal lesions. In the case of lichen nitidus, lesions often follow a geometric line due to the Köbner response, whereas physical trauma from scratching or injury causes lesions to form along the line of insult. Assessing patients for any newly initiated medications can help eliminate lichenoid drug eruptions. Lichen striatus perhaps has the most overlap with blaschkitis, having been described as also following the lines of Blaschko but occurring in children rather than adults. Inflammatory linear verrucous epidermal nevi also can be distinguished from blaschkitis on this premise, as these lesions arise during the first 5 years of life and generally affect the lower extremities.^4,5

Histopathology is somewhat variable but generally includes spongiotic dermatitis with concomitant interface
dermatitis characterized by T-cell infiltration. Spongiosis is a feature less commonly seen in lichen striatus. Lesions can progress over time from spongiotic dermatitis to spongiotic psoriasiform dermatitis and later to spongiotic psoriasiform lichenoid dermatitis.⁴ Treatment of blaschkitis should begin with reassurance of the benign nature of the dermatosis. Pruritic symptoms can be managed with a course of topical steroids.

Blaschkitis is a rare and self-limiting acquired dermatosis that should be incorporated into the differential diagnosis of Blaschko-linear dermatoses. Further investigation is needed to determine if blaschkitis and lichen striatus represent the ends of a disease spectrum or completely distinct entities.

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Frontal fibrosing alopecia (FFA) has been reported in association with lichen planus pigmentosus (LPP) and facial papules.^1-3 Lichen planus pigmentosus is a variant of lichen planus that causes hyperpigmentation of the face, neck, and/or intertriginous areas that may be useful as a clinical indicator in the development of FFA.¹ Facial papules in association with FFA are secondary to fibrosed vellus hairs.^2,3 Currently, reports of concomitant FFA, LPP, and facial papules in women with skin of color are limited in the literature. This case series includes 5 women of color (Hispanic and black) who presented to our clinic with FFA and various cutaneous associations. A review of the current literature on cutaneous associations of FFA also is provided.

Case Reports

Patient 1
A 50-year-old Hispanic woman who was previously presumed to have melasma by an outside physician presented with pruritus of the scalp and eyebrows of 1 month’s duration. Physical examination revealed decreased frontal scalp hair density with perifollicular erythema and scale with thinning of the lateral eyebrows. Hyperpigmented coalesced macules (Figure 1A) and erythematous perifollicular papules were noted along the temples and on the perioral skin. Depressed forehead and temporal veins also were noted (Figure 1B). A biopsy of the scalp demonstrated perifollicular and perivascular lymphocytic inflammation and fibrosed hair follicles, and a biopsy of the perioral skin demonstrated perivascular lymphocytic inflammation with melanophages in the papillary dermis. A diagnosis of FFA with LPP was established with these biopsies.

Patient 2
A 61-year-old black woman presented with asymptomatic hair loss along the frontal hairline for an unknown duration. On physical examination the frontal scalp and lateral eyebrows demonstrated decreased hair density with loss of follicular ostia. Fine, flesh-colored, monomorphic papules were scattered along the forehead and temples, and ill-defined brown pigmentation was present along the forehead, temples, and cheeks. Biopsy of the frontal scalp demonstrated patchy lichenoid inflammation with decreased number of follicles with replacement by follicular scars, confirming the diagnosis of FFA.

Patient 3
A 47-year-old Hispanic woman presented with hair loss of the frontal scalp and bilateral eyebrows with associated burning of 2 years’ duration. Physical examination demonstrated recession of the frontotemporal hairline with scattered lone hairs and thinning of the eyebrows. Innumerable flesh-colored papules were present on the forehead and temples (Figure 2A). Glabellar and eyebrow erythema was noted (Figure 2B). Biopsy of the frontal scalp demonstrated decreased terminal anagen hair follicles with perifollicular lymphoid infiltrate and fibrosis, consistent with a diagnosis of FFA. The patient was started on oral hydroxychloroquine 400 mg once daily, and 3 months later hyperpigmentation of the forehead and perioral skin was noted. The patient reported that she had facial hyperpigmentation prior to starting hydroxychloroquine and declined a biopsy.

Patient 4
A 40-year-old black woman presented with brown pruritic macles of the face, neck, arms, and forearms of 4 years’ duration. She also reported hair loss on the frontal and occipital scalp, eyebrows, and arms. On physical examination, ill-defined brown macules and patches were noted on the neck (Figure 3), face, arms, and forearms. Decreased hair density was noted on the frontal and occipital scalp with follicular dropout and perifollicular hyperpigmentation. Biopsy of the scalp demonstrated perivascular lymphocytic inflammation with sparse anagen follicles and fibrous tracts, and biopsy of the neck revealed superficial perivascular inflammation with numerous melanophages in the upper dermis; these histopathologic findings were consistent with FFA and LPP, respectively.

Patient 5
A 46-year-old black woman with history of hair loss presented with hyperpigmentation of the face and neck of 2 years’ duration. On physical examination decreased hair density of the frontal and vertex scalp and lateral eyebrows was noted. Flesh-colored papules were noted on the forehead and cheeks, and confluent dark brown patches were present on the temples and neck. Three punch biopsies were performed. Biopsy of the scalp revealed lymphocytic inflammation with surrounding fibroplasia with overlapping features of FFA and central centrifugal cicatricial alopecia (Figure 4). Biopsy of the neck revealed vacuolar interface dermatitis. Additionally, biopsy of a facial papule revealed lichenoid inflammation involving a vellus hair follicle. Clinical and histopathological correlation confirmed the diagnosis of FFA with LPP and facial papules.

Comment

Current understanding of FFA as a progressive, lymphocytic, scarring alopecia has expanded in recent years. Clinical observation suggests that the incidence of FFA is increasing⁴; however more epidemiologic data are needed. Frontal fibrosing alopecia presents clinically with symmetrical frontotemporal hair loss with lone hairs. Trichoscopy reveals perifollicular hyperkeratosis, perifollicular erythema, and follicular plugging in 72%, 66%, and 44% of cases, respectively.⁵ In one study (N=242), patients were classified into 3 clinical patterns of FFA: pattern I (linear) showed bandlike loss of frontal hair with normal density directly behind the hairline; pattern II (diffuse) showed loss of density behind the frontal hairline; and pattern III (double line) showed a pseudo–“fringe sign” appearance. The majority of patients were classified as either pattern I or II, with pattern II predicting a poorer prognosis.⁶

rontal fibrosing alopecia is increasingly recognized in men, with prevalence as high as 5%.¹ Facial hair involvement, particularly of the upper lip and sideburns, is an important consideration in men.⁷ Most studies suggest that 80% to 90% of affected women are postmenopausal,⁸ though a case series presented by Dlova¹ identified 27% of affected women as postmenopausal. The coexistence of premature menopause and hysterectomy in FFA patients suggests a hormonal contribution, but this association is still poorly understood.⁸ Epidemiologic data on ethnicity in FFA are sparse but suggest that white individuals are more likely to be affected. Frontal fibrosing alopecia also may be misdiagnosed as traction alopecia in Hispanic and black patients.⁸

It is prudent for physicians to assess for and recognize clinical clues to severe forms of FFA. A 2014 multicenter review of 355 patients identified 3 clinical entities that predicted more severe forms of FFA: eyelash loss (madarosis), loss of body hair, and facial papules.⁸ Madarosis occurs due to perifollicular inflammation and fibrosis of eyelash hair follicles. Similarly, perifollicular inflammation of body hair was present in 24% of patients (N=86), most commonly of the axillary and pubic hair. Facial papules form due to facial vellus hair inflammation and fibrosis and were identified in 14% of patients (N=49).⁸ These clinical findings may allow providers to predict more extensive clinical involvement of FFA.

Frontal fibrosing alopecia and LPP occur concomitantly in up 54% of patients, more commonly in darker-skinned patients.^1,9,10 Lichen planus pigmentosus frequently occurs on the face and neck, most commonly in a diffuse pattern, though reticulated and macular patterns also have been identified.¹¹ In some patients, LPP precedes the development of FFA and may be useful as a herald sign¹; therefore, it is important for dermatologists to evaluate for signs of FFA when evaluating those with LPP. Thorough evaluation in patients with skin of color also is important because FFA may be misdiagnosed as traction alopecia.

Additional cutaneous associations of FFA include eyebrow loss, glabellar red dots, and prominent frontal veins. Eyebrow loss occurs secondary to fibrosis of eyebrow hair follicles and has been found in 40% to 80% of patients with FFA; it is thought to be associated with milder forms of FFA.⁸ Glabellar red dots correlate with histopathologic lymphocytic inflammation of vellus hair follicles.¹² Additionally, frontal vein prominence has been described in FFA and is thought to be secondary to atrophy in this scarring process, perhaps worsened by local steroid treatments.¹³ Mucocutaneous lichen planus, rosacea, thyroid disease, vitiligo, and other autoimmune disorders also have been reported in patients with FFA.¹⁴

Conclusion

Concomitant FFA, LPP, and facial papules have been rarely reported and exemplify the spectrum of cutaneous associations with FFA, particularly in individuals with skin of color. Clinical variants and associations of FFA are broad, including predictors of poorer prognosis such as eyelash loss and vellus hair involvement seen as facial papules. Lichen planus pigmentosus is well described in association with FFA and may serve as a herald sign that frontal hair loss should not be mistaken for traction alopecia in early stages. Eyebrow loss is thought to represent milder disease. It is important for dermatologists to be aware of these findings to understand the breadth of this disease and for appropriate evaluation and management of patients with FFA.

Frontal fibrosing alopecia (FFA) has been reported in association with lichen planus pigmentosus (LPP) and facial papules.^1-3 Lichen planus pigmentosus is a variant of lichen planus that causes hyperpigmentation of the face, neck, and/or intertriginous areas that may be useful as a clinical indicator in the development of FFA.¹ Facial papules in association with FFA are secondary to fibrosed vellus hairs.^2,3 Currently, reports of concomitant FFA, LPP, and facial papules in women with skin of color are limited in the literature. This case series includes 5 women of color (Hispanic and black) who presented to our clinic with FFA and various cutaneous associations. A review of the current literature on cutaneous associations of FFA also is provided.

Case Reports

Patient 1
A 50-year-old Hispanic woman who was previously presumed to have melasma by an outside physician presented with pruritus of the scalp and eyebrows of 1 month’s duration. Physical examination revealed decreased frontal scalp hair density with perifollicular erythema and scale with thinning of the lateral eyebrows. Hyperpigmented coalesced macules (Figure 1A) and erythematous perifollicular papules were noted along the temples and on the perioral skin. Depressed forehead and temporal veins also were noted (Figure 1B). A biopsy of the scalp demonstrated perifollicular and perivascular lymphocytic inflammation and fibrosed hair follicles, and a biopsy of the perioral skin demonstrated perivascular lymphocytic inflammation with melanophages in the papillary dermis. A diagnosis of FFA with LPP was established with these biopsies.

Patient 2
A 61-year-old black woman presented with asymptomatic hair loss along the frontal hairline for an unknown duration. On physical examination the frontal scalp and lateral eyebrows demonstrated decreased hair density with loss of follicular ostia. Fine, flesh-colored, monomorphic papules were scattered along the forehead and temples, and ill-defined brown pigmentation was present along the forehead, temples, and cheeks. Biopsy of the frontal scalp demonstrated patchy lichenoid inflammation with decreased number of follicles with replacement by follicular scars, confirming the diagnosis of FFA.

Patient 3
A 47-year-old Hispanic woman presented with hair loss of the frontal scalp and bilateral eyebrows with associated burning of 2 years’ duration. Physical examination demonstrated recession of the frontotemporal hairline with scattered lone hairs and thinning of the eyebrows. Innumerable flesh-colored papules were present on the forehead and temples (Figure 2A). Glabellar and eyebrow erythema was noted (Figure 2B). Biopsy of the frontal scalp demonstrated decreased terminal anagen hair follicles with perifollicular lymphoid infiltrate and fibrosis, consistent with a diagnosis of FFA. The patient was started on oral hydroxychloroquine 400 mg once daily, and 3 months later hyperpigmentation of the forehead and perioral skin was noted. The patient reported that she had facial hyperpigmentation prior to starting hydroxychloroquine and declined a biopsy.

Patient 4
A 40-year-old black woman presented with brown pruritic macles of the face, neck, arms, and forearms of 4 years’ duration. She also reported hair loss on the frontal and occipital scalp, eyebrows, and arms. On physical examination, ill-defined brown macules and patches were noted on the neck (Figure 3), face, arms, and forearms. Decreased hair density was noted on the frontal and occipital scalp with follicular dropout and perifollicular hyperpigmentation. Biopsy of the scalp demonstrated perivascular lymphocytic inflammation with sparse anagen follicles and fibrous tracts, and biopsy of the neck revealed superficial perivascular inflammation with numerous melanophages in the upper dermis; these histopathologic findings were consistent with FFA and LPP, respectively.

Patient 5
A 46-year-old black woman with history of hair loss presented with hyperpigmentation of the face and neck of 2 years’ duration. On physical examination decreased hair density of the frontal and vertex scalp and lateral eyebrows was noted. Flesh-colored papules were noted on the forehead and cheeks, and confluent dark brown patches were present on the temples and neck. Three punch biopsies were performed. Biopsy of the scalp revealed lymphocytic inflammation with surrounding fibroplasia with overlapping features of FFA and central centrifugal cicatricial alopecia (Figure 4). Biopsy of the neck revealed vacuolar interface dermatitis. Additionally, biopsy of a facial papule revealed lichenoid inflammation involving a vellus hair follicle. Clinical and histopathological correlation confirmed the diagnosis of FFA with LPP and facial papules.

Comment

Current understanding of FFA as a progressive, lymphocytic, scarring alopecia has expanded in recent years. Clinical observation suggests that the incidence of FFA is increasing⁴; however more epidemiologic data are needed. Frontal fibrosing alopecia presents clinically with symmetrical frontotemporal hair loss with lone hairs. Trichoscopy reveals perifollicular hyperkeratosis, perifollicular erythema, and follicular plugging in 72%, 66%, and 44% of cases, respectively.⁵ In one study (N=242), patients were classified into 3 clinical patterns of FFA: pattern I (linear) showed bandlike loss of frontal hair with normal density directly behind the hairline; pattern II (diffuse) showed loss of density behind the frontal hairline; and pattern III (double line) showed a pseudo–“fringe sign” appearance. The majority of patients were classified as either pattern I or II, with pattern II predicting a poorer prognosis.⁶

rontal fibrosing alopecia is increasingly recognized in men, with prevalence as high as 5%.¹ Facial hair involvement, particularly of the upper lip and sideburns, is an important consideration in men.⁷ Most studies suggest that 80% to 90% of affected women are postmenopausal,⁸ though a case series presented by Dlova¹ identified 27% of affected women as postmenopausal. The coexistence of premature menopause and hysterectomy in FFA patients suggests a hormonal contribution, but this association is still poorly understood.⁸ Epidemiologic data on ethnicity in FFA are sparse but suggest that white individuals are more likely to be affected. Frontal fibrosing alopecia also may be misdiagnosed as traction alopecia in Hispanic and black patients.⁸

It is prudent for physicians to assess for and recognize clinical clues to severe forms of FFA. A 2014 multicenter review of 355 patients identified 3 clinical entities that predicted more severe forms of FFA: eyelash loss (madarosis), loss of body hair, and facial papules.⁸ Madarosis occurs due to perifollicular inflammation and fibrosis of eyelash hair follicles. Similarly, perifollicular inflammation of body hair was present in 24% of patients (N=86), most commonly of the axillary and pubic hair. Facial papules form due to facial vellus hair inflammation and fibrosis and were identified in 14% of patients (N=49).⁸ These clinical findings may allow providers to predict more extensive clinical involvement of FFA.

Frontal fibrosing alopecia and LPP occur concomitantly in up 54% of patients, more commonly in darker-skinned patients.^1,9,10 Lichen planus pigmentosus frequently occurs on the face and neck, most commonly in a diffuse pattern, though reticulated and macular patterns also have been identified.¹¹ In some patients, LPP precedes the development of FFA and may be useful as a herald sign¹; therefore, it is important for dermatologists to evaluate for signs of FFA when evaluating those with LPP. Thorough evaluation in patients with skin of color also is important because FFA may be misdiagnosed as traction alopecia.

Additional cutaneous associations of FFA include eyebrow loss, glabellar red dots, and prominent frontal veins. Eyebrow loss occurs secondary to fibrosis of eyebrow hair follicles and has been found in 40% to 80% of patients with FFA; it is thought to be associated with milder forms of FFA.⁸ Glabellar red dots correlate with histopathologic lymphocytic inflammation of vellus hair follicles.¹² Additionally, frontal vein prominence has been described in FFA and is thought to be secondary to atrophy in this scarring process, perhaps worsened by local steroid treatments.¹³ Mucocutaneous lichen planus, rosacea, thyroid disease, vitiligo, and other autoimmune disorders also have been reported in patients with FFA.¹⁴

Conclusion

Concomitant FFA, LPP, and facial papules have been rarely reported and exemplify the spectrum of cutaneous associations with FFA, particularly in individuals with skin of color. Clinical variants and associations of FFA are broad, including predictors of poorer prognosis such as eyelash loss and vellus hair involvement seen as facial papules. Lichen planus pigmentosus is well described in association with FFA and may serve as a herald sign that frontal hair loss should not be mistaken for traction alopecia in early stages. Eyebrow loss is thought to represent milder disease. It is important for dermatologists to be aware of these findings to understand the breadth of this disease and for appropriate evaluation and management of patients with FFA.

Frontal fibrosing alopecia (FFA) has been reported in association with lichen planus pigmentosus (LPP) and facial papules.^1-3 Lichen planus pigmentosus is a variant of lichen planus that causes hyperpigmentation of the face, neck, and/or intertriginous areas that may be useful as a clinical indicator in the development of FFA.¹ Facial papules in association with FFA are secondary to fibrosed vellus hairs.^2,3 Currently, reports of concomitant FFA, LPP, and facial papules in women with skin of color are limited in the literature. This case series includes 5 women of color (Hispanic and black) who presented to our clinic with FFA and various cutaneous associations. A review of the current literature on cutaneous associations of FFA also is provided.

Case Reports

Patient 1
A 50-year-old Hispanic woman who was previously presumed to have melasma by an outside physician presented with pruritus of the scalp and eyebrows of 1 month’s duration. Physical examination revealed decreased frontal scalp hair density with perifollicular erythema and scale with thinning of the lateral eyebrows. Hyperpigmented coalesced macules (Figure 1A) and erythematous perifollicular papules were noted along the temples and on the perioral skin. Depressed forehead and temporal veins also were noted (Figure 1B). A biopsy of the scalp demonstrated perifollicular and perivascular lymphocytic inflammation and fibrosed hair follicles, and a biopsy of the perioral skin demonstrated perivascular lymphocytic inflammation with melanophages in the papillary dermis. A diagnosis of FFA with LPP was established with these biopsies.

Patient 2
A 61-year-old black woman presented with asymptomatic hair loss along the frontal hairline for an unknown duration. On physical examination the frontal scalp and lateral eyebrows demonstrated decreased hair density with loss of follicular ostia. Fine, flesh-colored, monomorphic papules were scattered along the forehead and temples, and ill-defined brown pigmentation was present along the forehead, temples, and cheeks. Biopsy of the frontal scalp demonstrated patchy lichenoid inflammation with decreased number of follicles with replacement by follicular scars, confirming the diagnosis of FFA.

Patient 3
A 47-year-old Hispanic woman presented with hair loss of the frontal scalp and bilateral eyebrows with associated burning of 2 years’ duration. Physical examination demonstrated recession of the frontotemporal hairline with scattered lone hairs and thinning of the eyebrows. Innumerable flesh-colored papules were present on the forehead and temples (Figure 2A). Glabellar and eyebrow erythema was noted (Figure 2B). Biopsy of the frontal scalp demonstrated decreased terminal anagen hair follicles with perifollicular lymphoid infiltrate and fibrosis, consistent with a diagnosis of FFA. The patient was started on oral hydroxychloroquine 400 mg once daily, and 3 months later hyperpigmentation of the forehead and perioral skin was noted. The patient reported that she had facial hyperpigmentation prior to starting hydroxychloroquine and declined a biopsy.

Patient 4
A 40-year-old black woman presented with brown pruritic macles of the face, neck, arms, and forearms of 4 years’ duration. She also reported hair loss on the frontal and occipital scalp, eyebrows, and arms. On physical examination, ill-defined brown macules and patches were noted on the neck (Figure 3), face, arms, and forearms. Decreased hair density was noted on the frontal and occipital scalp with follicular dropout and perifollicular hyperpigmentation. Biopsy of the scalp demonstrated perivascular lymphocytic inflammation with sparse anagen follicles and fibrous tracts, and biopsy of the neck revealed superficial perivascular inflammation with numerous melanophages in the upper dermis; these histopathologic findings were consistent with FFA and LPP, respectively.

Patient 5
A 46-year-old black woman with history of hair loss presented with hyperpigmentation of the face and neck of 2 years’ duration. On physical examination decreased hair density of the frontal and vertex scalp and lateral eyebrows was noted. Flesh-colored papules were noted on the forehead and cheeks, and confluent dark brown patches were present on the temples and neck. Three punch biopsies were performed. Biopsy of the scalp revealed lymphocytic inflammation with surrounding fibroplasia with overlapping features of FFA and central centrifugal cicatricial alopecia (Figure 4). Biopsy of the neck revealed vacuolar interface dermatitis. Additionally, biopsy of a facial papule revealed lichenoid inflammation involving a vellus hair follicle. Clinical and histopathological correlation confirmed the diagnosis of FFA with LPP and facial papules.

Comment

Current understanding of FFA as a progressive, lymphocytic, scarring alopecia has expanded in recent years. Clinical observation suggests that the incidence of FFA is increasing⁴; however more epidemiologic data are needed. Frontal fibrosing alopecia presents clinically with symmetrical frontotemporal hair loss with lone hairs. Trichoscopy reveals perifollicular hyperkeratosis, perifollicular erythema, and follicular plugging in 72%, 66%, and 44% of cases, respectively.⁵ In one study (N=242), patients were classified into 3 clinical patterns of FFA: pattern I (linear) showed bandlike loss of frontal hair with normal density directly behind the hairline; pattern II (diffuse) showed loss of density behind the frontal hairline; and pattern III (double line) showed a pseudo–“fringe sign” appearance. The majority of patients were classified as either pattern I or II, with pattern II predicting a poorer prognosis.⁶

rontal fibrosing alopecia is increasingly recognized in men, with prevalence as high as 5%.¹ Facial hair involvement, particularly of the upper lip and sideburns, is an important consideration in men.⁷ Most studies suggest that 80% to 90% of affected women are postmenopausal,⁸ though a case series presented by Dlova¹ identified 27% of affected women as postmenopausal. The coexistence of premature menopause and hysterectomy in FFA patients suggests a hormonal contribution, but this association is still poorly understood.⁸ Epidemiologic data on ethnicity in FFA are sparse but suggest that white individuals are more likely to be affected. Frontal fibrosing alopecia also may be misdiagnosed as traction alopecia in Hispanic and black patients.⁸

It is prudent for physicians to assess for and recognize clinical clues to severe forms of FFA. A 2014 multicenter review of 355 patients identified 3 clinical entities that predicted more severe forms of FFA: eyelash loss (madarosis), loss of body hair, and facial papules.⁸ Madarosis occurs due to perifollicular inflammation and fibrosis of eyelash hair follicles. Similarly, perifollicular inflammation of body hair was present in 24% of patients (N=86), most commonly of the axillary and pubic hair. Facial papules form due to facial vellus hair inflammation and fibrosis and were identified in 14% of patients (N=49).⁸ These clinical findings may allow providers to predict more extensive clinical involvement of FFA.

Frontal fibrosing alopecia and LPP occur concomitantly in up 54% of patients, more commonly in darker-skinned patients.^1,9,10 Lichen planus pigmentosus frequently occurs on the face and neck, most commonly in a diffuse pattern, though reticulated and macular patterns also have been identified.¹¹ In some patients, LPP precedes the development of FFA and may be useful as a herald sign¹; therefore, it is important for dermatologists to evaluate for signs of FFA when evaluating those with LPP. Thorough evaluation in patients with skin of color also is important because FFA may be misdiagnosed as traction alopecia.

Additional cutaneous associations of FFA include eyebrow loss, glabellar red dots, and prominent frontal veins. Eyebrow loss occurs secondary to fibrosis of eyebrow hair follicles and has been found in 40% to 80% of patients with FFA; it is thought to be associated with milder forms of FFA.⁸ Glabellar red dots correlate with histopathologic lymphocytic inflammation of vellus hair follicles.¹² Additionally, frontal vein prominence has been described in FFA and is thought to be secondary to atrophy in this scarring process, perhaps worsened by local steroid treatments.¹³ Mucocutaneous lichen planus, rosacea, thyroid disease, vitiligo, and other autoimmune disorders also have been reported in patients with FFA.¹⁴

Conclusion

Concomitant FFA, LPP, and facial papules have been rarely reported and exemplify the spectrum of cutaneous associations with FFA, particularly in individuals with skin of color. Clinical variants and associations of FFA are broad, including predictors of poorer prognosis such as eyelash loss and vellus hair involvement seen as facial papules. Lichen planus pigmentosus is well described in association with FFA and may serve as a herald sign that frontal hair loss should not be mistaken for traction alopecia in early stages. Eyebrow loss is thought to represent milder disease. It is important for dermatologists to be aware of these findings to understand the breadth of this disease and for appropriate evaluation and management of patients with FFA.

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The Diagnosis: Terra Firma-Forme Dermatosis

Terra firma-forme dermatosis (TFFD), also known as Duncan dirty dermatosis, is an idiopathic benign cutaneous condition that is easily misdiagnosed or mismanaged. In 1987, Duncan et al1 first described the condition in children who had mothers that lamented over dirty skin spots that could not be washed off. The term terra firma translates in Latin to solid ground, which describes the characteristic dirtlike appearance of these lesions.

Terra firma-forme dermatosis most commonly affects children and young adults, though it can present in patients of any age without any known predisposing risk factors.^1-4 The lesions have a predilection for the face, neck, shoulders, trunk, and ankles. Terra firma-forme dermatosis has no association with bathing and hygiene habits, and most patients describe unsuccessful removal of the lesions, even after vigorous scrubbing with soaps and detergents at home. The lesions are asymptomatic, and many patients present to dermatology for cosmetic concerns.^1-8

The etiology of TFFD is not well understood and is considered a retention hyperkeratosis. Duncan et al¹ postulated that TFFD is the result of partial or improper maturation of keratinocytes leading to keratinocyte and melanin retention. Hematoxylin and eosin stains demonstrate lamellar hyperkeratosis of the stratum corneum without parakeratosis as well as keratin pearls scattered throughout. Mild acanthosis and papillomatosis also have been reported.^1,5-7 Fontana-Masson stain shows excess melanin in these lesions, extending from the basal layer to the stratum corneum. Fungal and bacterial stains as well as cultures often have no notable findings.^1,7 Similarly, histopathologic examination of our patient's biopsy with hematoxylin and eosin stain revealed hyperorthokeratosis with scattered naked vellus hair shafts and incidental yeast forms (Figure 1).

The differential diagnosis for TFFD may include pityriasis versicolor, confluent and reticulated papillomatosis, acanthosis nigricans, ichthyosis, malignant melanoma, and seborrheic keratosis. All of these diagnoses can be ruled out by the easy removal of the lesions with isopropyl alcohol 70%, which was performed on our patient by scrubbing the lesions with soaked gauze (Figure 2). Indeed, removal with isopropyl alcohol 70% is both the therapeutic and diagnostic procedure for TFFD.^1-8 Of note, dermatitis neglecta is histologically and clinically identical to TFFD, albeit with a history of uncleanly habits or exposure to dirty environments.

The diagnosis of TFFD often is discovered incidentally as physicians wipe the area with alcohol to prepare for biopsy.¹ Occasionally, vigorous scrubbing is needed to completely remove the lesions, and without this effort the lesions may be easily mistaken for another cutaneous process.³ Failure to consider TFFD as a diagnosis has led to unnecessary endocrine workups and invasive biopsies.⁴ Therefore, physicians should have early clinical suspicion of TFFD and be aware of the bedside diagnostic procedure using isopropyl alcohol.

The Diagnosis: Terra Firma-Forme Dermatosis

Terra firma-forme dermatosis (TFFD), also known as Duncan dirty dermatosis, is an idiopathic benign cutaneous condition that is easily misdiagnosed or mismanaged. In 1987, Duncan et al1 first described the condition in children who had mothers that lamented over dirty skin spots that could not be washed off. The term terra firma translates in Latin to solid ground, which describes the characteristic dirtlike appearance of these lesions.

Terra firma-forme dermatosis most commonly affects children and young adults, though it can present in patients of any age without any known predisposing risk factors.^1-4 The lesions have a predilection for the face, neck, shoulders, trunk, and ankles. Terra firma-forme dermatosis has no association with bathing and hygiene habits, and most patients describe unsuccessful removal of the lesions, even after vigorous scrubbing with soaps and detergents at home. The lesions are asymptomatic, and many patients present to dermatology for cosmetic concerns.^1-8

The etiology of TFFD is not well understood and is considered a retention hyperkeratosis. Duncan et al¹ postulated that TFFD is the result of partial or improper maturation of keratinocytes leading to keratinocyte and melanin retention. Hematoxylin and eosin stains demonstrate lamellar hyperkeratosis of the stratum corneum without parakeratosis as well as keratin pearls scattered throughout. Mild acanthosis and papillomatosis also have been reported.^1,5-7 Fontana-Masson stain shows excess melanin in these lesions, extending from the basal layer to the stratum corneum. Fungal and bacterial stains as well as cultures often have no notable findings.^1,7 Similarly, histopathologic examination of our patient's biopsy with hematoxylin and eosin stain revealed hyperorthokeratosis with scattered naked vellus hair shafts and incidental yeast forms (Figure 1).

The differential diagnosis for TFFD may include pityriasis versicolor, confluent and reticulated papillomatosis, acanthosis nigricans, ichthyosis, malignant melanoma, and seborrheic keratosis. All of these diagnoses can be ruled out by the easy removal of the lesions with isopropyl alcohol 70%, which was performed on our patient by scrubbing the lesions with soaked gauze (Figure 2). Indeed, removal with isopropyl alcohol 70% is both the therapeutic and diagnostic procedure for TFFD.^1-8 Of note, dermatitis neglecta is histologically and clinically identical to TFFD, albeit with a history of uncleanly habits or exposure to dirty environments.

The diagnosis of TFFD often is discovered incidentally as physicians wipe the area with alcohol to prepare for biopsy.¹ Occasionally, vigorous scrubbing is needed to completely remove the lesions, and without this effort the lesions may be easily mistaken for another cutaneous process.³ Failure to consider TFFD as a diagnosis has led to unnecessary endocrine workups and invasive biopsies.⁴ Therefore, physicians should have early clinical suspicion of TFFD and be aware of the bedside diagnostic procedure using isopropyl alcohol.

The Diagnosis: Terra Firma-Forme Dermatosis

Terra firma-forme dermatosis (TFFD), also known as Duncan dirty dermatosis, is an idiopathic benign cutaneous condition that is easily misdiagnosed or mismanaged. In 1987, Duncan et al1 first described the condition in children who had mothers that lamented over dirty skin spots that could not be washed off. The term terra firma translates in Latin to solid ground, which describes the characteristic dirtlike appearance of these lesions.

Terra firma-forme dermatosis most commonly affects children and young adults, though it can present in patients of any age without any known predisposing risk factors.^1-4 The lesions have a predilection for the face, neck, shoulders, trunk, and ankles. Terra firma-forme dermatosis has no association with bathing and hygiene habits, and most patients describe unsuccessful removal of the lesions, even after vigorous scrubbing with soaps and detergents at home. The lesions are asymptomatic, and many patients present to dermatology for cosmetic concerns.^1-8

The etiology of TFFD is not well understood and is considered a retention hyperkeratosis. Duncan et al¹ postulated that TFFD is the result of partial or improper maturation of keratinocytes leading to keratinocyte and melanin retention. Hematoxylin and eosin stains demonstrate lamellar hyperkeratosis of the stratum corneum without parakeratosis as well as keratin pearls scattered throughout. Mild acanthosis and papillomatosis also have been reported.^1,5-7 Fontana-Masson stain shows excess melanin in these lesions, extending from the basal layer to the stratum corneum. Fungal and bacterial stains as well as cultures often have no notable findings.^1,7 Similarly, histopathologic examination of our patient's biopsy with hematoxylin and eosin stain revealed hyperorthokeratosis with scattered naked vellus hair shafts and incidental yeast forms (Figure 1).

The differential diagnosis for TFFD may include pityriasis versicolor, confluent and reticulated papillomatosis, acanthosis nigricans, ichthyosis, malignant melanoma, and seborrheic keratosis. All of these diagnoses can be ruled out by the easy removal of the lesions with isopropyl alcohol 70%, which was performed on our patient by scrubbing the lesions with soaked gauze (Figure 2). Indeed, removal with isopropyl alcohol 70% is both the therapeutic and diagnostic procedure for TFFD.^1-8 Of note, dermatitis neglecta is histologically and clinically identical to TFFD, albeit with a history of uncleanly habits or exposure to dirty environments.

The diagnosis of TFFD often is discovered incidentally as physicians wipe the area with alcohol to prepare for biopsy.¹ Occasionally, vigorous scrubbing is needed to completely remove the lesions, and without this effort the lesions may be easily mistaken for another cutaneous process.³ Failure to consider TFFD as a diagnosis has led to unnecessary endocrine workups and invasive biopsies.⁴ Therefore, physicians should have early clinical suspicion of TFFD and be aware of the bedside diagnostic procedure using isopropyl alcohol.

Vitiligo is a disfiguring skin condition that is thought to result from autoimmune destruction of melanocytes in the skin, leading to patchy depigmentation. The prevalence of vitiligo is estimated at 1% worldwide.¹ Once seen as merely a cosmetic disorder, it is increasingly recognized for its devastating psychological effects. As skin quality, texture, and color are a few of the first things people notice about others, skin plays a major role in our daily interactions with the world. Vitiligo often affects the face and other visible areas of the body; thus, it is associated with impaired quality of life, and affected individuals often experience psychosocial impairment including anxiety, depression, stigmatization, and self-harm ideation.² Indeed, vitiligo is a condition with not only a visible skin component but a deeper psychological component that also is important to recognize and address. However, due in large part to recent exposure to vitiligo through mainstream media, general understanding about and attitudes toward this condition are changing. As a result, vitiligo has seen a surge in outreach by those affected by the disease.

Perhaps the most well-known current face of vitiligo is Chantelle Brown-Young, a black fashion model, activist, and vitiligo spokesperson known professionally as Winnie Harlow. Diagnosed with vitiligo in childhood, she revealed she was teased and bullied and at one point contemplated suicide. “The continuous harassment and the despair that [vitiligo] brought on my life was so unbearably dehumanizing that I wanted to kill myself,” she disclosed.³ After competing on America’s Next Top Model in 2014, Winnie Harlow became a household name for redefining global standards of beauty and, in her own words, accepting the differences that make us unique and authentic.⁴ She went on to speak at the Dove Self-Esteem Project panel at the 2015 Women in the World London Summit and was presented with the Role Model award at the Portuguese GQ Men of the Year event that same year.⁵

More recently, Amy Deanna, a model with vitiligo, was featured in videos for CoverGirl’s 2018 “I Am What I Make Up” campaign in which she is shown enhancing her various skin tones rather than hiding them by applying both light and dark shades of makeup on her face. In a press release she stated, “Vitiligo awareness is something that is very important to me. Being given a platform to [raise awareness] means so much.”⁶

Additionally, Brock Elbank, a London-based photographer, recently launched a photograph series of men and women with vitiligo on the digital platform Instagram.⁷ In a recent interview he stated, “I see beauty in what many see as different. Unique individuals who stand out from the crowd are what inspire me to do what I do.”⁷

Lee Thomas, a television broadcaster and author of the book Turning White: A Memoir of Change is yet another example of a vitiligo patient who recently stopped hiding his condition. He admitted he has had people refuse to shake his hand due to his condition but has used the experience to educate others. He stated, “Because I’m in this position, I think this is where my next thing is supposed to be. It’s supposed to be about sharing and helping, and hopefully leaving the planet a little better for everybody else who comes along with vitiligo.”⁸ Thomas is dedicated to inspiring others with the condition and started the Clarity Lee Thomas Foundation to provide emotional and mental support to those with vitiligo.

Critics may say this vitiligo movement is merely another example of exploitation of what is unique or different by mainstream media and the fashion industry, similar to prior movements for plus-sized models, natural hairstyles in black women, and transgender identification. Even if partially true, the ultimate effect has been an increase in attention and representation of individuals with vitiligo in mainstream media. At the time this article was being published (September 2018), an Instagram search for #vitiligo yielded approximately 226,000 posts. For comparison with other much more common dermatologic conditions, #eczema returned approximately 958,000 results, #moles returned approximately 65,000 results, and #skincancer returned approximately 104,000 results. Additionally, the Vitiligo Research Foundation currently has more than 5000 followers on Instagram, which is as many as the Melanoma Research Foundation and almost twice as many as the Skin Cancer Foundation, supporting the idea that mainstream representation of individuals with vitiligo is contributing to raising awareness and backing of organizations aimed at making advancements in this area of dermatology.

As more individuals gain an understanding and curiosity about this disease, perhaps more research and investigation will be done to improve treatment options and outcomes for patients with vitiligo. With this movement, perhaps vitiligo patients will feel more comfortable and confident in their skin.

Vitiligo is a disfiguring skin condition that is thought to result from autoimmune destruction of melanocytes in the skin, leading to patchy depigmentation. The prevalence of vitiligo is estimated at 1% worldwide.¹ Once seen as merely a cosmetic disorder, it is increasingly recognized for its devastating psychological effects. As skin quality, texture, and color are a few of the first things people notice about others, skin plays a major role in our daily interactions with the world. Vitiligo often affects the face and other visible areas of the body; thus, it is associated with impaired quality of life, and affected individuals often experience psychosocial impairment including anxiety, depression, stigmatization, and self-harm ideation.² Indeed, vitiligo is a condition with not only a visible skin component but a deeper psychological component that also is important to recognize and address. However, due in large part to recent exposure to vitiligo through mainstream media, general understanding about and attitudes toward this condition are changing. As a result, vitiligo has seen a surge in outreach by those affected by the disease.

Perhaps the most well-known current face of vitiligo is Chantelle Brown-Young, a black fashion model, activist, and vitiligo spokesperson known professionally as Winnie Harlow. Diagnosed with vitiligo in childhood, she revealed she was teased and bullied and at one point contemplated suicide. “The continuous harassment and the despair that [vitiligo] brought on my life was so unbearably dehumanizing that I wanted to kill myself,” she disclosed.³ After competing on America’s Next Top Model in 2014, Winnie Harlow became a household name for redefining global standards of beauty and, in her own words, accepting the differences that make us unique and authentic.⁴ She went on to speak at the Dove Self-Esteem Project panel at the 2015 Women in the World London Summit and was presented with the Role Model award at the Portuguese GQ Men of the Year event that same year.⁵

More recently, Amy Deanna, a model with vitiligo, was featured in videos for CoverGirl’s 2018 “I Am What I Make Up” campaign in which she is shown enhancing her various skin tones rather than hiding them by applying both light and dark shades of makeup on her face. In a press release she stated, “Vitiligo awareness is something that is very important to me. Being given a platform to [raise awareness] means so much.”⁶

Additionally, Brock Elbank, a London-based photographer, recently launched a photograph series of men and women with vitiligo on the digital platform Instagram.⁷ In a recent interview he stated, “I see beauty in what many see as different. Unique individuals who stand out from the crowd are what inspire me to do what I do.”⁷

Lee Thomas, a television broadcaster and author of the book Turning White: A Memoir of Change is yet another example of a vitiligo patient who recently stopped hiding his condition. He admitted he has had people refuse to shake his hand due to his condition but has used the experience to educate others. He stated, “Because I’m in this position, I think this is where my next thing is supposed to be. It’s supposed to be about sharing and helping, and hopefully leaving the planet a little better for everybody else who comes along with vitiligo.”⁸ Thomas is dedicated to inspiring others with the condition and started the Clarity Lee Thomas Foundation to provide emotional and mental support to those with vitiligo.

Critics may say this vitiligo movement is merely another example of exploitation of what is unique or different by mainstream media and the fashion industry, similar to prior movements for plus-sized models, natural hairstyles in black women, and transgender identification. Even if partially true, the ultimate effect has been an increase in attention and representation of individuals with vitiligo in mainstream media. At the time this article was being published (September 2018), an Instagram search for #vitiligo yielded approximately 226,000 posts. For comparison with other much more common dermatologic conditions, #eczema returned approximately 958,000 results, #moles returned approximately 65,000 results, and #skincancer returned approximately 104,000 results. Additionally, the Vitiligo Research Foundation currently has more than 5000 followers on Instagram, which is as many as the Melanoma Research Foundation and almost twice as many as the Skin Cancer Foundation, supporting the idea that mainstream representation of individuals with vitiligo is contributing to raising awareness and backing of organizations aimed at making advancements in this area of dermatology.

As more individuals gain an understanding and curiosity about this disease, perhaps more research and investigation will be done to improve treatment options and outcomes for patients with vitiligo. With this movement, perhaps vitiligo patients will feel more comfortable and confident in their skin.

Vitiligo is a disfiguring skin condition that is thought to result from autoimmune destruction of melanocytes in the skin, leading to patchy depigmentation. The prevalence of vitiligo is estimated at 1% worldwide.¹ Once seen as merely a cosmetic disorder, it is increasingly recognized for its devastating psychological effects. As skin quality, texture, and color are a few of the first things people notice about others, skin plays a major role in our daily interactions with the world. Vitiligo often affects the face and other visible areas of the body; thus, it is associated with impaired quality of life, and affected individuals often experience psychosocial impairment including anxiety, depression, stigmatization, and self-harm ideation.² Indeed, vitiligo is a condition with not only a visible skin component but a deeper psychological component that also is important to recognize and address. However, due in large part to recent exposure to vitiligo through mainstream media, general understanding about and attitudes toward this condition are changing. As a result, vitiligo has seen a surge in outreach by those affected by the disease.

Perhaps the most well-known current face of vitiligo is Chantelle Brown-Young, a black fashion model, activist, and vitiligo spokesperson known professionally as Winnie Harlow. Diagnosed with vitiligo in childhood, she revealed she was teased and bullied and at one point contemplated suicide. “The continuous harassment and the despair that [vitiligo] brought on my life was so unbearably dehumanizing that I wanted to kill myself,” she disclosed.³ After competing on America’s Next Top Model in 2014, Winnie Harlow became a household name for redefining global standards of beauty and, in her own words, accepting the differences that make us unique and authentic.⁴ She went on to speak at the Dove Self-Esteem Project panel at the 2015 Women in the World London Summit and was presented with the Role Model award at the Portuguese GQ Men of the Year event that same year.⁵

More recently, Amy Deanna, a model with vitiligo, was featured in videos for CoverGirl’s 2018 “I Am What I Make Up” campaign in which she is shown enhancing her various skin tones rather than hiding them by applying both light and dark shades of makeup on her face. In a press release she stated, “Vitiligo awareness is something that is very important to me. Being given a platform to [raise awareness] means so much.”⁶

Additionally, Brock Elbank, a London-based photographer, recently launched a photograph series of men and women with vitiligo on the digital platform Instagram.⁷ In a recent interview he stated, “I see beauty in what many see as different. Unique individuals who stand out from the crowd are what inspire me to do what I do.”⁷

Lee Thomas, a television broadcaster and author of the book Turning White: A Memoir of Change is yet another example of a vitiligo patient who recently stopped hiding his condition. He admitted he has had people refuse to shake his hand due to his condition but has used the experience to educate others. He stated, “Because I’m in this position, I think this is where my next thing is supposed to be. It’s supposed to be about sharing and helping, and hopefully leaving the planet a little better for everybody else who comes along with vitiligo.”⁸ Thomas is dedicated to inspiring others with the condition and started the Clarity Lee Thomas Foundation to provide emotional and mental support to those with vitiligo.

Critics may say this vitiligo movement is merely another example of exploitation of what is unique or different by mainstream media and the fashion industry, similar to prior movements for plus-sized models, natural hairstyles in black women, and transgender identification. Even if partially true, the ultimate effect has been an increase in attention and representation of individuals with vitiligo in mainstream media. At the time this article was being published (September 2018), an Instagram search for #vitiligo yielded approximately 226,000 posts. For comparison with other much more common dermatologic conditions, #eczema returned approximately 958,000 results, #moles returned approximately 65,000 results, and #skincancer returned approximately 104,000 results. Additionally, the Vitiligo Research Foundation currently has more than 5000 followers on Instagram, which is as many as the Melanoma Research Foundation and almost twice as many as the Skin Cancer Foundation, supporting the idea that mainstream representation of individuals with vitiligo is contributing to raising awareness and backing of organizations aimed at making advancements in this area of dermatology.

As more individuals gain an understanding and curiosity about this disease, perhaps more research and investigation will be done to improve treatment options and outcomes for patients with vitiligo. With this movement, perhaps vitiligo patients will feel more comfortable and confident in their skin.

Nevus of Ota, originally referred to as nevus fusco-caeruleus ophthalmomaxillaris, initially was described in 1939 by Ota and Tanino.¹ It is a dermal melanocytic hamartoma arising from incomplete migration of neural crest melanocytes to the epidermis during embryogenesis, resulting in nesting of subtle bands of dendritic melanocytes in the upper dermis. More common in Asians, Native Americans, and females, this hyperpigmented dermatosis most often is unilaterally distributed along the ophthalmic (V1) and maxillary (V2) branches of the trigeminal nerve.² In some patients, nevus of Ota also is associated with ocular, orbital, and leptomeningeal melanocytosis. Approximately 15% of nevi of Ota have an activating guanine nucleotide-binding protein G(q) subunit alpha (GNAQ) or G protein subunit alpha 11 (GNAQ) mutation; 85% of uveal melanomas harbor one of these mutations.³ Although uncommon, neoplastic transformation with extension or metastasis to the brain has been reported in patients with nevus of Ota.⁴

We report the case of a 29-year-old woman with a long-standing history of nevus of Ota who presented acutely with an intracranial melanoma as an extension of a primary uveal melanoma.

Case Report

A 29-year-old woman with a history of a nevus of Ota involving the left inner canthus, eyelids, sclera, and superior malar cheek that had been present since birth presented to the emergency department with an acute onset of severe headache, blurred vision, and vomiting. Computed tomography (CT) and magnetic resonance imaging of the brain revealed a hemorrhagic mass in the left frontal lobe. Subsequent frontal craniotomy and resection revealed an intracranial melanoma.

Two weeks following surgery, the patient underwent magnetic resonance imaging and combined positron emission tomography and CT scans that demonstrated a fluorodeoxyglucose-avid left retro-orbital mass. Histopathology of a biopsy from the left retro-orbital mass that had been obtained intraoperatively demonstrated a pigmented, spindled to epithelioid neoplasm with areas of marked atypia and a high mitotic rate that was compatible with malignant melanoma (Figure 1). Intracranial biopsies were sent for genetic study and were found to harbor GNAQ (Q209P) and BRCA1-associated protein 1 (BAP1)(p.P324fs*11) mutations.

The patient was referred to dermatology by neurosurgery for evaluation of a suspected primary cutaneous melanoma. Biopsies of 2 blue papules that had appeared over the last 2 years within the nevus of Ota on the left medial canthus and left malar cheek (Figure 2) revealed cellular blue nevi (Figure 3). No primary cutaneous melanoma was identified. Based on the genetic profile described above and the presence of GNAQ and BAP1 mutations, the patient was referred to ophthalmology. Inferotemporal darkening of the choroid, most likely consistent with a primary uveal melanoma, was discovered. The intracranial melanoma was thought to have arisen from the primary uveal melanoma.

The patient entered a clinical trial at an outside institution several weeks after initial presentation to our institution for treatment with a mitogen-activated protein kinase MEK1 inhibitor as well as radiation therapy. The patient was lost to follow-up.

Comment

It has been demonstrated that homozygous loss of BAP1, located on the chromosome 3p21.1 locus, allows for progression to metastatic disease in uveal melanoma. The BAP1 gene codes for ubiquitin carboxyl-terminal hydrolase 7, which is involved in the removal of ubiquitin from proteins. This enzyme binds to BRCA1 (BRCA1, DNA repair associated) via the RING (Really Interesting New Gene) finger domain and acts as a tumor suppressor.⁵ Biallelic BAP1 mutations allow the transition to malignancy in concert with other mutations, such as GNAQ. Identification of a BAP1 mutation may serve as a valuable diagnostic and future therapeutic target in uveal melanoma.

Currently, there are no drugs that directly target mutated GNA11 and GNAQ proteins. Because aberrant GNA11 and GNAQ proteins activate MEK1, several MEK1 inhibitors are being tested with the hope of achieving indirect suppression of GNA11/GNAQ.⁶

We present a rare case of BAP1 and GNAQ mutations in intracranial melanoma associated with nevus of Ota. Although the uveal melanoma was not confirmed on histopathology, the clear mention of foci within the eye by ophthalmology, positron emission tomography–CT scan showing a fluorodeoxyglucose-avid left retro-orbital mass, and genetic studies of the intracranial biopsies were highly suggestive of a primary uveal melanoma.

Our case highlights the importance of ongoing ocular screening in patients with nevus of Ota, noting the possibility of malignant transformation. Furthermore, patients with nevus of Ota with ocular involvement may benefit from testing of BAP1 protein expression by immunohistochemistry.⁷ Identification of BAP1 and GNAQ mutations in patients with nevus of Ota place them at markedly higher risk for malignant melanoma. Therefore, dermatologic evaluation of patients with nevus of Ota should include a thorough review of the patient’s history and skin examination as well as referral for ophthalmologic evaluation.

Nevus of Ota, originally referred to as nevus fusco-caeruleus ophthalmomaxillaris, initially was described in 1939 by Ota and Tanino.¹ It is a dermal melanocytic hamartoma arising from incomplete migration of neural crest melanocytes to the epidermis during embryogenesis, resulting in nesting of subtle bands of dendritic melanocytes in the upper dermis. More common in Asians, Native Americans, and females, this hyperpigmented dermatosis most often is unilaterally distributed along the ophthalmic (V1) and maxillary (V2) branches of the trigeminal nerve.² In some patients, nevus of Ota also is associated with ocular, orbital, and leptomeningeal melanocytosis. Approximately 15% of nevi of Ota have an activating guanine nucleotide-binding protein G(q) subunit alpha (GNAQ) or G protein subunit alpha 11 (GNAQ) mutation; 85% of uveal melanomas harbor one of these mutations.³ Although uncommon, neoplastic transformation with extension or metastasis to the brain has been reported in patients with nevus of Ota.⁴

We report the case of a 29-year-old woman with a long-standing history of nevus of Ota who presented acutely with an intracranial melanoma as an extension of a primary uveal melanoma.

Case Report

A 29-year-old woman with a history of a nevus of Ota involving the left inner canthus, eyelids, sclera, and superior malar cheek that had been present since birth presented to the emergency department with an acute onset of severe headache, blurred vision, and vomiting. Computed tomography (CT) and magnetic resonance imaging of the brain revealed a hemorrhagic mass in the left frontal lobe. Subsequent frontal craniotomy and resection revealed an intracranial melanoma.

Two weeks following surgery, the patient underwent magnetic resonance imaging and combined positron emission tomography and CT scans that demonstrated a fluorodeoxyglucose-avid left retro-orbital mass. Histopathology of a biopsy from the left retro-orbital mass that had been obtained intraoperatively demonstrated a pigmented, spindled to epithelioid neoplasm with areas of marked atypia and a high mitotic rate that was compatible with malignant melanoma (Figure 1). Intracranial biopsies were sent for genetic study and were found to harbor GNAQ (Q209P) and BRCA1-associated protein 1 (BAP1)(p.P324fs*11) mutations.

The patient was referred to dermatology by neurosurgery for evaluation of a suspected primary cutaneous melanoma. Biopsies of 2 blue papules that had appeared over the last 2 years within the nevus of Ota on the left medial canthus and left malar cheek (Figure 2) revealed cellular blue nevi (Figure 3). No primary cutaneous melanoma was identified. Based on the genetic profile described above and the presence of GNAQ and BAP1 mutations, the patient was referred to ophthalmology. Inferotemporal darkening of the choroid, most likely consistent with a primary uveal melanoma, was discovered. The intracranial melanoma was thought to have arisen from the primary uveal melanoma.

The patient entered a clinical trial at an outside institution several weeks after initial presentation to our institution for treatment with a mitogen-activated protein kinase MEK1 inhibitor as well as radiation therapy. The patient was lost to follow-up.

Comment

It has been demonstrated that homozygous loss of BAP1, located on the chromosome 3p21.1 locus, allows for progression to metastatic disease in uveal melanoma. The BAP1 gene codes for ubiquitin carboxyl-terminal hydrolase 7, which is involved in the removal of ubiquitin from proteins. This enzyme binds to BRCA1 (BRCA1, DNA repair associated) via the RING (Really Interesting New Gene) finger domain and acts as a tumor suppressor.⁵ Biallelic BAP1 mutations allow the transition to malignancy in concert with other mutations, such as GNAQ. Identification of a BAP1 mutation may serve as a valuable diagnostic and future therapeutic target in uveal melanoma.

Currently, there are no drugs that directly target mutated GNA11 and GNAQ proteins. Because aberrant GNA11 and GNAQ proteins activate MEK1, several MEK1 inhibitors are being tested with the hope of achieving indirect suppression of GNA11/GNAQ.⁶

We present a rare case of BAP1 and GNAQ mutations in intracranial melanoma associated with nevus of Ota. Although the uveal melanoma was not confirmed on histopathology, the clear mention of foci within the eye by ophthalmology, positron emission tomography–CT scan showing a fluorodeoxyglucose-avid left retro-orbital mass, and genetic studies of the intracranial biopsies were highly suggestive of a primary uveal melanoma.

Our case highlights the importance of ongoing ocular screening in patients with nevus of Ota, noting the possibility of malignant transformation. Furthermore, patients with nevus of Ota with ocular involvement may benefit from testing of BAP1 protein expression by immunohistochemistry.⁷ Identification of BAP1 and GNAQ mutations in patients with nevus of Ota place them at markedly higher risk for malignant melanoma. Therefore, dermatologic evaluation of patients with nevus of Ota should include a thorough review of the patient’s history and skin examination as well as referral for ophthalmologic evaluation.

Nevus of Ota, originally referred to as nevus fusco-caeruleus ophthalmomaxillaris, initially was described in 1939 by Ota and Tanino.¹ It is a dermal melanocytic hamartoma arising from incomplete migration of neural crest melanocytes to the epidermis during embryogenesis, resulting in nesting of subtle bands of dendritic melanocytes in the upper dermis. More common in Asians, Native Americans, and females, this hyperpigmented dermatosis most often is unilaterally distributed along the ophthalmic (V1) and maxillary (V2) branches of the trigeminal nerve.² In some patients, nevus of Ota also is associated with ocular, orbital, and leptomeningeal melanocytosis. Approximately 15% of nevi of Ota have an activating guanine nucleotide-binding protein G(q) subunit alpha (GNAQ) or G protein subunit alpha 11 (GNAQ) mutation; 85% of uveal melanomas harbor one of these mutations.³ Although uncommon, neoplastic transformation with extension or metastasis to the brain has been reported in patients with nevus of Ota.⁴

We report the case of a 29-year-old woman with a long-standing history of nevus of Ota who presented acutely with an intracranial melanoma as an extension of a primary uveal melanoma.

Case Report

A 29-year-old woman with a history of a nevus of Ota involving the left inner canthus, eyelids, sclera, and superior malar cheek that had been present since birth presented to the emergency department with an acute onset of severe headache, blurred vision, and vomiting. Computed tomography (CT) and magnetic resonance imaging of the brain revealed a hemorrhagic mass in the left frontal lobe. Subsequent frontal craniotomy and resection revealed an intracranial melanoma.

Two weeks following surgery, the patient underwent magnetic resonance imaging and combined positron emission tomography and CT scans that demonstrated a fluorodeoxyglucose-avid left retro-orbital mass. Histopathology of a biopsy from the left retro-orbital mass that had been obtained intraoperatively demonstrated a pigmented, spindled to epithelioid neoplasm with areas of marked atypia and a high mitotic rate that was compatible with malignant melanoma (Figure 1). Intracranial biopsies were sent for genetic study and were found to harbor GNAQ (Q209P) and BRCA1-associated protein 1 (BAP1)(p.P324fs*11) mutations.

The patient was referred to dermatology by neurosurgery for evaluation of a suspected primary cutaneous melanoma. Biopsies of 2 blue papules that had appeared over the last 2 years within the nevus of Ota on the left medial canthus and left malar cheek (Figure 2) revealed cellular blue nevi (Figure 3). No primary cutaneous melanoma was identified. Based on the genetic profile described above and the presence of GNAQ and BAP1 mutations, the patient was referred to ophthalmology. Inferotemporal darkening of the choroid, most likely consistent with a primary uveal melanoma, was discovered. The intracranial melanoma was thought to have arisen from the primary uveal melanoma.

The patient entered a clinical trial at an outside institution several weeks after initial presentation to our institution for treatment with a mitogen-activated protein kinase MEK1 inhibitor as well as radiation therapy. The patient was lost to follow-up.

Comment

It has been demonstrated that homozygous loss of BAP1, located on the chromosome 3p21.1 locus, allows for progression to metastatic disease in uveal melanoma. The BAP1 gene codes for ubiquitin carboxyl-terminal hydrolase 7, which is involved in the removal of ubiquitin from proteins. This enzyme binds to BRCA1 (BRCA1, DNA repair associated) via the RING (Really Interesting New Gene) finger domain and acts as a tumor suppressor.⁵ Biallelic BAP1 mutations allow the transition to malignancy in concert with other mutations, such as GNAQ. Identification of a BAP1 mutation may serve as a valuable diagnostic and future therapeutic target in uveal melanoma.

Currently, there are no drugs that directly target mutated GNA11 and GNAQ proteins. Because aberrant GNA11 and GNAQ proteins activate MEK1, several MEK1 inhibitors are being tested with the hope of achieving indirect suppression of GNA11/GNAQ.⁶

We present a rare case of BAP1 and GNAQ mutations in intracranial melanoma associated with nevus of Ota. Although the uveal melanoma was not confirmed on histopathology, the clear mention of foci within the eye by ophthalmology, positron emission tomography–CT scan showing a fluorodeoxyglucose-avid left retro-orbital mass, and genetic studies of the intracranial biopsies were highly suggestive of a primary uveal melanoma.

Our case highlights the importance of ongoing ocular screening in patients with nevus of Ota, noting the possibility of malignant transformation. Furthermore, patients with nevus of Ota with ocular involvement may benefit from testing of BAP1 protein expression by immunohistochemistry.⁷ Identification of BAP1 and GNAQ mutations in patients with nevus of Ota place them at markedly higher risk for malignant melanoma. Therefore, dermatologic evaluation of patients with nevus of Ota should include a thorough review of the patient’s history and skin examination as well as referral for ophthalmologic evaluation.

SAN DIEGO – According to J. Stuart Nelson, MD, PhD, three general principles guide the laser treatment of vascular skin lesions. The first is to target blood vessels beneath the surface of the skin.

“You’re going to be using wavelengths of light generally in the green and yellow portion of the spectrum,” Dr. Nelson, professor of surgery and biomedical engineering at the Beckman Laser Institute and Medical Clinic at the University of California, Irvine, said at the annual Masters of Aesthetics Symposium. “Blue light is highly absorbed by hemoglobin but unfortunately, blue light is highly scattered by human skin, so it won’t penetrate deep enough into the dermis. So primarily, we’re targeting hemoglobin using green and yellow light sources.”

The second principle is to match the pulse width with the vessel size, while the third is to give sufficient energy to irreversibly injure vessels based on selective photothermolysis.

Next, he advised clinicians to ask themselves three questions: What is the vessel size? “The larger the vessel, the longer the thermal relaxation time,” he said. What is the vessel depth? Deeper vessels require longer wavelengths of light and larger spot sizes. What is the patient’s skin phototype? Darker skin contains more epidermal melanin and requires extra caution during treatment.

Dr. Nelson listed seven optimal devices for the treatment of vascular skin lesions: intense pulsed light (IPL) with wavelengths of 515-1,200 nm and pulse durations of 1-10 ms, pulsed green light with a wavelength of 532 nm and pulse durations of 1-50 ms, pulsed dye yellow light with wavelengths of 585-600 nm and pulse durations of 0.5-40 ms, pulsed dye plus Nd:YAG with wavelengths of 595 and 1,064 nm and pulse durations of 0.5-40 ms, alexandrite laser with a wavelength of 755 nm and pulse durations of 0.25-100 ms, diode laser with a wavelength of 940 nm and pulse durations of 5-100 ms, and the pulsed Nd:YAG laser with a wavelength of 1,064 nm and pulse durations of 0.25-100 ms.

“You can get good results with every one of these devices,” Dr. Nelson said. “What you need to do is pick one and become what R. Rox Anderson, MD, calls an ‘endpointologist,’ so you can understand the clinical endpoints. Do not use a cookbook approach by trying to memorize treatment settings.”

Pulsed dye lasers with a wavelength of 585-600 nm have been the standard of care for years, he said, and is the treatment of choice for port wine stains in infants and young children. Upsides include the ability to treat large areas quickly and the ability to use two to three separate passes. It also induces diminution in diffuse redness and telangiectasia. Drawbacks include its potential to cause purpura when short pulse durations are used, it requires several treatments, it can be painful, and it causes considerable edema and erythema.

Millisecond green lasers at a wavelength of 532 nm are also effective for treating vascular skin lesions. “The nice thing about these devices is that you can focus them down to very small spots, so you can literally trace out individual blood vessels,” Dr. Nelson said. Other upsides include the fact that it can be performed without producing purpura, only transient erythema if few areas are treated. Drawbacks are that it’s moderately painful and may cause considerable edema. It also causes significant melanin absorption so is not advised for use in tanned and darker-skinned individuals. For all patients, contact cooling must be assured.

IPL, meanwhile, “can be very useful for treating not only vascular lesions, but also concurrently pigmented lesions such as poikiloderma of Civatte,” Dr. Nelson said. Potential drawbacks to IPL therapy are that the spectrum of light emitted and the pulse duration characteristics vary between devices and multiple treatments are required.

Finally, in the millisecond domain, the pulsed alexandrite 755-nm and Nd:YAG 1,064-nm lasers “are very good when trying to target something very deep in the skin like a vein,” he said. “But when you’re using those devices, you’re coagulating a large volume of tissue, so you need to be very careful about the amount of heat that you’re generating deep in the skin.”

When consulting with patients who have rosacea or telangiectasia, Dr. Nelson tells them multiple treatments will be required. “These are chronic conditions, and they may need ongoing maintenance treatments. The nice thing about all these procedures you’re doing for rosacea and telangiectasia is that they can be combined with all of your FDA [Food and Drug Administration]-approved topical and oral treatment protocols. All of these drugs you have at your disposal to medically treat rosacea can be all used concurrently with your laser treatment. When you see a patient you need to emphasize to them: ‘I’m not treating your rosacea with the laser. I’m treating a symptom of your rosacea with the laser.’ ”

Dr. Nelson closed his presentation by offering basic principles for success, the first being do no harm. “That’s the single most important thing you want to remember. No one will get mad if the blood vessel’s still there, but they’ll get very mad if something bad happens. You also want to underpromise and overdeliver. I always tell patients it’s going to require two to four treatments. When in doubt, don’t treat or undertreat. You can always treat again.”

If you’re concerned, perform a test spot. “There’s nothing wrong with that, particularly in a patient where you’re not sure what the outcome will be,” he said. “Check for any unusual skin reaction and for potential success of the procedure. Finally, don’t treat patients who are tanned.”

Dr. Nelson reported having intellectual property rights with Syneron Candela.

dbrunk@mdedge.com

SAN DIEGO – According to J. Stuart Nelson, MD, PhD, three general principles guide the laser treatment of vascular skin lesions. The first is to target blood vessels beneath the surface of the skin.

“You’re going to be using wavelengths of light generally in the green and yellow portion of the spectrum,” Dr. Nelson, professor of surgery and biomedical engineering at the Beckman Laser Institute and Medical Clinic at the University of California, Irvine, said at the annual Masters of Aesthetics Symposium. “Blue light is highly absorbed by hemoglobin but unfortunately, blue light is highly scattered by human skin, so it won’t penetrate deep enough into the dermis. So primarily, we’re targeting hemoglobin using green and yellow light sources.”

The second principle is to match the pulse width with the vessel size, while the third is to give sufficient energy to irreversibly injure vessels based on selective photothermolysis.

Next, he advised clinicians to ask themselves three questions: What is the vessel size? “The larger the vessel, the longer the thermal relaxation time,” he said. What is the vessel depth? Deeper vessels require longer wavelengths of light and larger spot sizes. What is the patient’s skin phototype? Darker skin contains more epidermal melanin and requires extra caution during treatment.

Dr. Nelson listed seven optimal devices for the treatment of vascular skin lesions: intense pulsed light (IPL) with wavelengths of 515-1,200 nm and pulse durations of 1-10 ms, pulsed green light with a wavelength of 532 nm and pulse durations of 1-50 ms, pulsed dye yellow light with wavelengths of 585-600 nm and pulse durations of 0.5-40 ms, pulsed dye plus Nd:YAG with wavelengths of 595 and 1,064 nm and pulse durations of 0.5-40 ms, alexandrite laser with a wavelength of 755 nm and pulse durations of 0.25-100 ms, diode laser with a wavelength of 940 nm and pulse durations of 5-100 ms, and the pulsed Nd:YAG laser with a wavelength of 1,064 nm and pulse durations of 0.25-100 ms.

“You can get good results with every one of these devices,” Dr. Nelson said. “What you need to do is pick one and become what R. Rox Anderson, MD, calls an ‘endpointologist,’ so you can understand the clinical endpoints. Do not use a cookbook approach by trying to memorize treatment settings.”

Pulsed dye lasers with a wavelength of 585-600 nm have been the standard of care for years, he said, and is the treatment of choice for port wine stains in infants and young children. Upsides include the ability to treat large areas quickly and the ability to use two to three separate passes. It also induces diminution in diffuse redness and telangiectasia. Drawbacks include its potential to cause purpura when short pulse durations are used, it requires several treatments, it can be painful, and it causes considerable edema and erythema.

Millisecond green lasers at a wavelength of 532 nm are also effective for treating vascular skin lesions. “The nice thing about these devices is that you can focus them down to very small spots, so you can literally trace out individual blood vessels,” Dr. Nelson said. Other upsides include the fact that it can be performed without producing purpura, only transient erythema if few areas are treated. Drawbacks are that it’s moderately painful and may cause considerable edema. It also causes significant melanin absorption so is not advised for use in tanned and darker-skinned individuals. For all patients, contact cooling must be assured.

IPL, meanwhile, “can be very useful for treating not only vascular lesions, but also concurrently pigmented lesions such as poikiloderma of Civatte,” Dr. Nelson said. Potential drawbacks to IPL therapy are that the spectrum of light emitted and the pulse duration characteristics vary between devices and multiple treatments are required.

Finally, in the millisecond domain, the pulsed alexandrite 755-nm and Nd:YAG 1,064-nm lasers “are very good when trying to target something very deep in the skin like a vein,” he said. “But when you’re using those devices, you’re coagulating a large volume of tissue, so you need to be very careful about the amount of heat that you’re generating deep in the skin.”

When consulting with patients who have rosacea or telangiectasia, Dr. Nelson tells them multiple treatments will be required. “These are chronic conditions, and they may need ongoing maintenance treatments. The nice thing about all these procedures you’re doing for rosacea and telangiectasia is that they can be combined with all of your FDA [Food and Drug Administration]-approved topical and oral treatment protocols. All of these drugs you have at your disposal to medically treat rosacea can be all used concurrently with your laser treatment. When you see a patient you need to emphasize to them: ‘I’m not treating your rosacea with the laser. I’m treating a symptom of your rosacea with the laser.’ ”

Dr. Nelson closed his presentation by offering basic principles for success, the first being do no harm. “That’s the single most important thing you want to remember. No one will get mad if the blood vessel’s still there, but they’ll get very mad if something bad happens. You also want to underpromise and overdeliver. I always tell patients it’s going to require two to four treatments. When in doubt, don’t treat or undertreat. You can always treat again.”

If you’re concerned, perform a test spot. “There’s nothing wrong with that, particularly in a patient where you’re not sure what the outcome will be,” he said. “Check for any unusual skin reaction and for potential success of the procedure. Finally, don’t treat patients who are tanned.”

Dr. Nelson reported having intellectual property rights with Syneron Candela.

dbrunk@mdedge.com

SAN DIEGO – According to J. Stuart Nelson, MD, PhD, three general principles guide the laser treatment of vascular skin lesions. The first is to target blood vessels beneath the surface of the skin.

“You’re going to be using wavelengths of light generally in the green and yellow portion of the spectrum,” Dr. Nelson, professor of surgery and biomedical engineering at the Beckman Laser Institute and Medical Clinic at the University of California, Irvine, said at the annual Masters of Aesthetics Symposium. “Blue light is highly absorbed by hemoglobin but unfortunately, blue light is highly scattered by human skin, so it won’t penetrate deep enough into the dermis. So primarily, we’re targeting hemoglobin using green and yellow light sources.”

The second principle is to match the pulse width with the vessel size, while the third is to give sufficient energy to irreversibly injure vessels based on selective photothermolysis.

Next, he advised clinicians to ask themselves three questions: What is the vessel size? “The larger the vessel, the longer the thermal relaxation time,” he said. What is the vessel depth? Deeper vessels require longer wavelengths of light and larger spot sizes. What is the patient’s skin phototype? Darker skin contains more epidermal melanin and requires extra caution during treatment.

Dr. Nelson listed seven optimal devices for the treatment of vascular skin lesions: intense pulsed light (IPL) with wavelengths of 515-1,200 nm and pulse durations of 1-10 ms, pulsed green light with a wavelength of 532 nm and pulse durations of 1-50 ms, pulsed dye yellow light with wavelengths of 585-600 nm and pulse durations of 0.5-40 ms, pulsed dye plus Nd:YAG with wavelengths of 595 and 1,064 nm and pulse durations of 0.5-40 ms, alexandrite laser with a wavelength of 755 nm and pulse durations of 0.25-100 ms, diode laser with a wavelength of 940 nm and pulse durations of 5-100 ms, and the pulsed Nd:YAG laser with a wavelength of 1,064 nm and pulse durations of 0.25-100 ms.

“You can get good results with every one of these devices,” Dr. Nelson said. “What you need to do is pick one and become what R. Rox Anderson, MD, calls an ‘endpointologist,’ so you can understand the clinical endpoints. Do not use a cookbook approach by trying to memorize treatment settings.”

Pulsed dye lasers with a wavelength of 585-600 nm have been the standard of care for years, he said, and is the treatment of choice for port wine stains in infants and young children. Upsides include the ability to treat large areas quickly and the ability to use two to three separate passes. It also induces diminution in diffuse redness and telangiectasia. Drawbacks include its potential to cause purpura when short pulse durations are used, it requires several treatments, it can be painful, and it causes considerable edema and erythema.

Millisecond green lasers at a wavelength of 532 nm are also effective for treating vascular skin lesions. “The nice thing about these devices is that you can focus them down to very small spots, so you can literally trace out individual blood vessels,” Dr. Nelson said. Other upsides include the fact that it can be performed without producing purpura, only transient erythema if few areas are treated. Drawbacks are that it’s moderately painful and may cause considerable edema. It also causes significant melanin absorption so is not advised for use in tanned and darker-skinned individuals. For all patients, contact cooling must be assured.

IPL, meanwhile, “can be very useful for treating not only vascular lesions, but also concurrently pigmented lesions such as poikiloderma of Civatte,” Dr. Nelson said. Potential drawbacks to IPL therapy are that the spectrum of light emitted and the pulse duration characteristics vary between devices and multiple treatments are required.

Finally, in the millisecond domain, the pulsed alexandrite 755-nm and Nd:YAG 1,064-nm lasers “are very good when trying to target something very deep in the skin like a vein,” he said. “But when you’re using those devices, you’re coagulating a large volume of tissue, so you need to be very careful about the amount of heat that you’re generating deep in the skin.”

When consulting with patients who have rosacea or telangiectasia, Dr. Nelson tells them multiple treatments will be required. “These are chronic conditions, and they may need ongoing maintenance treatments. The nice thing about all these procedures you’re doing for rosacea and telangiectasia is that they can be combined with all of your FDA [Food and Drug Administration]-approved topical and oral treatment protocols. All of these drugs you have at your disposal to medically treat rosacea can be all used concurrently with your laser treatment. When you see a patient you need to emphasize to them: ‘I’m not treating your rosacea with the laser. I’m treating a symptom of your rosacea with the laser.’ ”

Dr. Nelson closed his presentation by offering basic principles for success, the first being do no harm. “That’s the single most important thing you want to remember. No one will get mad if the blood vessel’s still there, but they’ll get very mad if something bad happens. You also want to underpromise and overdeliver. I always tell patients it’s going to require two to four treatments. When in doubt, don’t treat or undertreat. You can always treat again.”

If you’re concerned, perform a test spot. “There’s nothing wrong with that, particularly in a patient where you’re not sure what the outcome will be,” he said. “Check for any unusual skin reaction and for potential success of the procedure. Finally, don’t treat patients who are tanned.”

Dr. Nelson reported having intellectual property rights with Syneron Candela.

dbrunk@mdedge.com

To the Editor:

A 17-year-old adolescent boy presented with dark spots on the legs and back of 2 months’ duration. He was not taking any medications and the spots could not be washed away by scrubbing with soap and water. He denied symptoms, except occasional itching. Family history revealed a maternal uncle with protein C deficiency and a maternal grandmother with systemic lupus erythematosus. Review of systems was negative; the patient denied joint pain and contact with heating pads or laptop computers. Based on the initial presentation, an underlying systemic condition was suspected. Physical examination revealed reticulate, nonblanching, brown patches on the bilateral arms, legs, and back in an apparent livedoid pattern (Figure). The patient’s history and physical examination suggested terra firma-forme dermatosis, livedo racemosa, or another vasculopathic process. However, gentle rubbing of the skin with an alcohol swab removed the discoloration completely, leading to the diagnosis of terra firma-forme dermatosis.

Livedo racemosa appears as an irregular, focal, reticulated discoloration of the skin.¹ The reticulated pattern of livedo racemosa has a branched or broken-up appearance.² Livedo racemosa indicates a disruption in the vasculature due to inflammation or occlusion.¹ The change is pathologic and does not blanch or resolve with warming.^1,2 The condition can progress to pigmentation and ulceration.¹ Livedo racemosa is a cutaneous manifestation of underlying vascular pathology. Due to a variety of causes, skin biopsy is nondiagnostic. Livedo racemosa can be caused by conditions such as systemic lupus erythematosus, syphilis, tuberculosis, polycythemia rubra vera, and Sneddon syndrome, among others.^3-5

Terra firma-forme dermatosis was reported in 1987 by Duncan et al.⁶ The condition classically presents with an exasperated mother who is unable to clean the “dirt” off her child’s skin despite multiple vigorous scrubbing attempts. The condition most commonly occurs in the summer months on the neck, face, and ankles.^7,8 Duncan et al⁶ reported that when the affected area was prepared for a biopsy, clean skin was revealed after wiping with an alcohol swab. No other cleansing agent has been reported to effectively remove the discoloration of terra firma-forme dermatosis. Hoping to elucidate a cause, Duncan et al⁶ performed both bacteriologic and fungal studies. The bacterial skin culture grew only normal flora, and fungal culture grew only normal contaminants consistent with the potassium hydroxide preparation of skin scraping. Histopathologic examination showed hyperkeratosis and orthokeratosis but not parakeratosis. Staining revealed melanin in the hyperkeratotic areas.⁶ Although the cause of this condition largely is unknown, it is thought that the epidermis in the affected areas could undergo altered maturation, resulting in trapping melanin that causes the skin to appear hyperkeratotic and hyperpigmented.¹ In our case, wiping the skin revealed the unsuspected diagnosis of terra firma-forme dermatosis displaying an unusual pseudolivedoid pattern. With apparently hyperpigmented processes, rubbing the skin with alcohol may help avoid unnecessary aggressive workup.

To the Editor:

A 17-year-old adolescent boy presented with dark spots on the legs and back of 2 months’ duration. He was not taking any medications and the spots could not be washed away by scrubbing with soap and water. He denied symptoms, except occasional itching. Family history revealed a maternal uncle with protein C deficiency and a maternal grandmother with systemic lupus erythematosus. Review of systems was negative; the patient denied joint pain and contact with heating pads or laptop computers. Based on the initial presentation, an underlying systemic condition was suspected. Physical examination revealed reticulate, nonblanching, brown patches on the bilateral arms, legs, and back in an apparent livedoid pattern (Figure). The patient’s history and physical examination suggested terra firma-forme dermatosis, livedo racemosa, or another vasculopathic process. However, gentle rubbing of the skin with an alcohol swab removed the discoloration completely, leading to the diagnosis of terra firma-forme dermatosis.

Livedo racemosa appears as an irregular, focal, reticulated discoloration of the skin.¹ The reticulated pattern of livedo racemosa has a branched or broken-up appearance.² Livedo racemosa indicates a disruption in the vasculature due to inflammation or occlusion.¹ The change is pathologic and does not blanch or resolve with warming.^1,2 The condition can progress to pigmentation and ulceration.¹ Livedo racemosa is a cutaneous manifestation of underlying vascular pathology. Due to a variety of causes, skin biopsy is nondiagnostic. Livedo racemosa can be caused by conditions such as systemic lupus erythematosus, syphilis, tuberculosis, polycythemia rubra vera, and Sneddon syndrome, among others.^3-5

Terra firma-forme dermatosis was reported in 1987 by Duncan et al.⁶ The condition classically presents with an exasperated mother who is unable to clean the “dirt” off her child’s skin despite multiple vigorous scrubbing attempts. The condition most commonly occurs in the summer months on the neck, face, and ankles.^7,8 Duncan et al⁶ reported that when the affected area was prepared for a biopsy, clean skin was revealed after wiping with an alcohol swab. No other cleansing agent has been reported to effectively remove the discoloration of terra firma-forme dermatosis. Hoping to elucidate a cause, Duncan et al⁶ performed both bacteriologic and fungal studies. The bacterial skin culture grew only normal flora, and fungal culture grew only normal contaminants consistent with the potassium hydroxide preparation of skin scraping. Histopathologic examination showed hyperkeratosis and orthokeratosis but not parakeratosis. Staining revealed melanin in the hyperkeratotic areas.⁶ Although the cause of this condition largely is unknown, it is thought that the epidermis in the affected areas could undergo altered maturation, resulting in trapping melanin that causes the skin to appear hyperkeratotic and hyperpigmented.¹ In our case, wiping the skin revealed the unsuspected diagnosis of terra firma-forme dermatosis displaying an unusual pseudolivedoid pattern. With apparently hyperpigmented processes, rubbing the skin with alcohol may help avoid unnecessary aggressive workup.

To the Editor:

A 17-year-old adolescent boy presented with dark spots on the legs and back of 2 months’ duration. He was not taking any medications and the spots could not be washed away by scrubbing with soap and water. He denied symptoms, except occasional itching. Family history revealed a maternal uncle with protein C deficiency and a maternal grandmother with systemic lupus erythematosus. Review of systems was negative; the patient denied joint pain and contact with heating pads or laptop computers. Based on the initial presentation, an underlying systemic condition was suspected. Physical examination revealed reticulate, nonblanching, brown patches on the bilateral arms, legs, and back in an apparent livedoid pattern (Figure). The patient’s history and physical examination suggested terra firma-forme dermatosis, livedo racemosa, or another vasculopathic process. However, gentle rubbing of the skin with an alcohol swab removed the discoloration completely, leading to the diagnosis of terra firma-forme dermatosis.

Livedo racemosa appears as an irregular, focal, reticulated discoloration of the skin.¹ The reticulated pattern of livedo racemosa has a branched or broken-up appearance.² Livedo racemosa indicates a disruption in the vasculature due to inflammation or occlusion.¹ The change is pathologic and does not blanch or resolve with warming.^1,2 The condition can progress to pigmentation and ulceration.¹ Livedo racemosa is a cutaneous manifestation of underlying vascular pathology. Due to a variety of causes, skin biopsy is nondiagnostic. Livedo racemosa can be caused by conditions such as systemic lupus erythematosus, syphilis, tuberculosis, polycythemia rubra vera, and Sneddon syndrome, among others.^3-5

Terra firma-forme dermatosis was reported in 1987 by Duncan et al.⁶ The condition classically presents with an exasperated mother who is unable to clean the “dirt” off her child’s skin despite multiple vigorous scrubbing attempts. The condition most commonly occurs in the summer months on the neck, face, and ankles.^7,8 Duncan et al⁶ reported that when the affected area was prepared for a biopsy, clean skin was revealed after wiping with an alcohol swab. No other cleansing agent has been reported to effectively remove the discoloration of terra firma-forme dermatosis. Hoping to elucidate a cause, Duncan et al⁶ performed both bacteriologic and fungal studies. The bacterial skin culture grew only normal flora, and fungal culture grew only normal contaminants consistent with the potassium hydroxide preparation of skin scraping. Histopathologic examination showed hyperkeratosis and orthokeratosis but not parakeratosis. Staining revealed melanin in the hyperkeratotic areas.⁶ Although the cause of this condition largely is unknown, it is thought that the epidermis in the affected areas could undergo altered maturation, resulting in trapping melanin that causes the skin to appear hyperkeratotic and hyperpigmented.¹ In our case, wiping the skin revealed the unsuspected diagnosis of terra firma-forme dermatosis displaying an unusual pseudolivedoid pattern. With apparently hyperpigmented processes, rubbing the skin with alcohol may help avoid unnecessary aggressive workup.

Extending oral propranolol treatment up to 12 months of age increased the success rate for high-risk infantile hemangioma, according to results published in Pediatrics.

Previous studies of oral propranol for infantile hemangiomas (IH) have revealed its efficacy, although there is no consensus on the optimal treatment duration. Nonetheless, treatment up to 12 months of age has been proposed if patients don’t respond after 6 months. Infants with high-risk hemangiomas, however, have been excluded from previous studies, authors of the current study explained.

In an open-label study of patients aged 35-150 days the success rate of oral propranolol was 47% after 6 months of treatment. The rate increased to 76% after the initial treatment period, reported Eulalia Baselga, MD, of the department of dermatology at Hospital de la Santa Creu i Sant Pau in Barcelona, and coauthors.

Investigators studied 45 patients from 10 hospitals in Spain and Poland between June 2015 and February 2017. The patients had high-risk IH in the proliferative phase. High-risk hemangiomas were defined as those that were life threatening, at risk for functional impact, disfiguring, or ulcerated nonresponsive to standard wound care measures.

Oral propranolol was administered twice daily at a dosage of 3 mg/kg per day. During the initial treatment period (ITP), patients received propranolol for a minimum of 6 months, and if treatment was not successful, it continued until success or up to 12 months of age.

Patients who achieved success in the initial phase were managed for 3 months with no treatment, and if there was rebound growth, treatment was restarted for up to 6 months at the provider’s discretion.

Treatment was considered a success if the target hemangioma resolved and there was no functional impact. The IH was considered resolved if it disappeared, even if there were minimal telangiectasias, erythema, skin thickening, soft tissue swelling, or the presence of sequelae.

Treatment success was achieved by 21 (47%) patients after 6 months and by 34 (76%) patients by the end of the ITP. Functional impact was determined using the Hemangioma Severity and Hemangioma Dynamic Complication scales. Adverse events, reported by 80% of patients, were resolved by the end of the study and included respiratory syncytial virus bronchiolitis, ulcerated hemangioma, pneumonia and respiratory failure, inguinal hernia, upper respiratory tract infection, dehydration, bronchitis, choking, and thermal burn. Although no patients experienced adverse events that resulted in discontinuation of treatment, 35 events led to temporary discontinuation, primarily due to respiratory events, the authors reported.

The results indicate that “oral propranolol is effective in treating high-risk IH with a favorable safety profile,” the authors concluded.

The study was funded by the Institut de Recherche Pierre Fabre Several authors were employed by or had other relationships with Pierre Fabre. The other authors had no conflicts of interest.

SOURCE: Baselga E et al. Pediatrics. 2018. doi: 10.1542/peds.2017-3866.

Extending oral propranolol treatment up to 12 months of age increased the success rate for high-risk infantile hemangioma, according to results published in Pediatrics.

Previous studies of oral propranol for infantile hemangiomas (IH) have revealed its efficacy, although there is no consensus on the optimal treatment duration. Nonetheless, treatment up to 12 months of age has been proposed if patients don’t respond after 6 months. Infants with high-risk hemangiomas, however, have been excluded from previous studies, authors of the current study explained.

In an open-label study of patients aged 35-150 days the success rate of oral propranolol was 47% after 6 months of treatment. The rate increased to 76% after the initial treatment period, reported Eulalia Baselga, MD, of the department of dermatology at Hospital de la Santa Creu i Sant Pau in Barcelona, and coauthors.

Investigators studied 45 patients from 10 hospitals in Spain and Poland between June 2015 and February 2017. The patients had high-risk IH in the proliferative phase. High-risk hemangiomas were defined as those that were life threatening, at risk for functional impact, disfiguring, or ulcerated nonresponsive to standard wound care measures.

Oral propranolol was administered twice daily at a dosage of 3 mg/kg per day. During the initial treatment period (ITP), patients received propranolol for a minimum of 6 months, and if treatment was not successful, it continued until success or up to 12 months of age.

Patients who achieved success in the initial phase were managed for 3 months with no treatment, and if there was rebound growth, treatment was restarted for up to 6 months at the provider’s discretion.

Treatment was considered a success if the target hemangioma resolved and there was no functional impact. The IH was considered resolved if it disappeared, even if there were minimal telangiectasias, erythema, skin thickening, soft tissue swelling, or the presence of sequelae.

Treatment success was achieved by 21 (47%) patients after 6 months and by 34 (76%) patients by the end of the ITP. Functional impact was determined using the Hemangioma Severity and Hemangioma Dynamic Complication scales. Adverse events, reported by 80% of patients, were resolved by the end of the study and included respiratory syncytial virus bronchiolitis, ulcerated hemangioma, pneumonia and respiratory failure, inguinal hernia, upper respiratory tract infection, dehydration, bronchitis, choking, and thermal burn. Although no patients experienced adverse events that resulted in discontinuation of treatment, 35 events led to temporary discontinuation, primarily due to respiratory events, the authors reported.

The results indicate that “oral propranolol is effective in treating high-risk IH with a favorable safety profile,” the authors concluded.

The study was funded by the Institut de Recherche Pierre Fabre Several authors were employed by or had other relationships with Pierre Fabre. The other authors had no conflicts of interest.

SOURCE: Baselga E et al. Pediatrics. 2018. doi: 10.1542/peds.2017-3866.

Extending oral propranolol treatment up to 12 months of age increased the success rate for high-risk infantile hemangioma, according to results published in Pediatrics.

Previous studies of oral propranol for infantile hemangiomas (IH) have revealed its efficacy, although there is no consensus on the optimal treatment duration. Nonetheless, treatment up to 12 months of age has been proposed if patients don’t respond after 6 months. Infants with high-risk hemangiomas, however, have been excluded from previous studies, authors of the current study explained.

In an open-label study of patients aged 35-150 days the success rate of oral propranolol was 47% after 6 months of treatment. The rate increased to 76% after the initial treatment period, reported Eulalia Baselga, MD, of the department of dermatology at Hospital de la Santa Creu i Sant Pau in Barcelona, and coauthors.

Investigators studied 45 patients from 10 hospitals in Spain and Poland between June 2015 and February 2017. The patients had high-risk IH in the proliferative phase. High-risk hemangiomas were defined as those that were life threatening, at risk for functional impact, disfiguring, or ulcerated nonresponsive to standard wound care measures.

Oral propranolol was administered twice daily at a dosage of 3 mg/kg per day. During the initial treatment period (ITP), patients received propranolol for a minimum of 6 months, and if treatment was not successful, it continued until success or up to 12 months of age.

Patients who achieved success in the initial phase were managed for 3 months with no treatment, and if there was rebound growth, treatment was restarted for up to 6 months at the provider’s discretion.

Treatment was considered a success if the target hemangioma resolved and there was no functional impact. The IH was considered resolved if it disappeared, even if there were minimal telangiectasias, erythema, skin thickening, soft tissue swelling, or the presence of sequelae.

Treatment success was achieved by 21 (47%) patients after 6 months and by 34 (76%) patients by the end of the ITP. Functional impact was determined using the Hemangioma Severity and Hemangioma Dynamic Complication scales. Adverse events, reported by 80% of patients, were resolved by the end of the study and included respiratory syncytial virus bronchiolitis, ulcerated hemangioma, pneumonia and respiratory failure, inguinal hernia, upper respiratory tract infection, dehydration, bronchitis, choking, and thermal burn. Although no patients experienced adverse events that resulted in discontinuation of treatment, 35 events led to temporary discontinuation, primarily due to respiratory events, the authors reported.

The results indicate that “oral propranolol is effective in treating high-risk IH with a favorable safety profile,” the authors concluded.

The study was funded by the Institut de Recherche Pierre Fabre Several authors were employed by or had other relationships with Pierre Fabre. The other authors had no conflicts of interest.

SOURCE: Baselga E et al. Pediatrics. 2018. doi: 10.1542/peds.2017-3866.