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A 56-year-old black woman presented with asymptomatic hypopigmented macules on her back, chest, face, and lateral arms
They often coalesce near the midline, and occasionally extend beyond the trunk to the arms, legs, head, or neck. PMH is more frequently diagnosed among black individuals, although it affects all races and ethnicities. The natural history of PMH can vary from stable and progressive disease, and may resolve spontaneously after a few years. The pathogenesis of PMH remains unknown. It has been proposed that the hypopigmentation is caused by decreased melanin production and altered melanosome dispersal in reaction to Propionibacterium acnes.
PMH must be distinguished from some of its clinical mimickers, including vitiligo, hypopigmented mycosis fungoides, tinea versicolor, and pityriasis alba. Potassium hydroxide preparations can be performed in the office to evaluate for tinea versicolor. An additional tool to aid in diagnosis is the use of a Wood’s light. The lesions of PMH characteristically show punctiform orange-red follicular fluorescence when exposed to a Wood’s light, indicating the presence of a porphyrin-producing organism, presumably P. acnes. A skin biopsy is necessary to rule out hypopigmented mycosis fungoides.
Skin biopsy of PMH typically reveals decreased melanin with a normal number of melanocytes. In our patient, a punch biopsy of the right lateral arm demonstrated minimally decreased density of epidermal melanocytes with dermal pigment incontinence. SOX10 immunohistochemical staining demonstrated scattered melanocytes in the epidermis. Preserved melanin within keratinocytes was noted.
In our patient, there was significant spread to the face, which is highly unusual and has only been documented in a few case series. There are no standard recommendations for definitive treatment of PMH. Topical antimicrobial therapies, such as clindamycin solution and benzoyl peroxide gel, have been beneficial in some studies. Tetracyclines, narrow-band ultraviolet B phototherapy, and even isotretinoin have had some reported success.
This case and photo were submitted by Mr. Franzetti, Dr. Rush, and Dr. Shalin of the University of Arkansas for Medical Sciences, Little Rock.
Donna Bilu Martin, MD, is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to dermnews@mdedge.com.
They often coalesce near the midline, and occasionally extend beyond the trunk to the arms, legs, head, or neck. PMH is more frequently diagnosed among black individuals, although it affects all races and ethnicities. The natural history of PMH can vary from stable and progressive disease, and may resolve spontaneously after a few years. The pathogenesis of PMH remains unknown. It has been proposed that the hypopigmentation is caused by decreased melanin production and altered melanosome dispersal in reaction to Propionibacterium acnes.
PMH must be distinguished from some of its clinical mimickers, including vitiligo, hypopigmented mycosis fungoides, tinea versicolor, and pityriasis alba. Potassium hydroxide preparations can be performed in the office to evaluate for tinea versicolor. An additional tool to aid in diagnosis is the use of a Wood’s light. The lesions of PMH characteristically show punctiform orange-red follicular fluorescence when exposed to a Wood’s light, indicating the presence of a porphyrin-producing organism, presumably P. acnes. A skin biopsy is necessary to rule out hypopigmented mycosis fungoides.
Skin biopsy of PMH typically reveals decreased melanin with a normal number of melanocytes. In our patient, a punch biopsy of the right lateral arm demonstrated minimally decreased density of epidermal melanocytes with dermal pigment incontinence. SOX10 immunohistochemical staining demonstrated scattered melanocytes in the epidermis. Preserved melanin within keratinocytes was noted.
In our patient, there was significant spread to the face, which is highly unusual and has only been documented in a few case series. There are no standard recommendations for definitive treatment of PMH. Topical antimicrobial therapies, such as clindamycin solution and benzoyl peroxide gel, have been beneficial in some studies. Tetracyclines, narrow-band ultraviolet B phototherapy, and even isotretinoin have had some reported success.
This case and photo were submitted by Mr. Franzetti, Dr. Rush, and Dr. Shalin of the University of Arkansas for Medical Sciences, Little Rock.
Donna Bilu Martin, MD, is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to dermnews@mdedge.com.
They often coalesce near the midline, and occasionally extend beyond the trunk to the arms, legs, head, or neck. PMH is more frequently diagnosed among black individuals, although it affects all races and ethnicities. The natural history of PMH can vary from stable and progressive disease, and may resolve spontaneously after a few years. The pathogenesis of PMH remains unknown. It has been proposed that the hypopigmentation is caused by decreased melanin production and altered melanosome dispersal in reaction to Propionibacterium acnes.
PMH must be distinguished from some of its clinical mimickers, including vitiligo, hypopigmented mycosis fungoides, tinea versicolor, and pityriasis alba. Potassium hydroxide preparations can be performed in the office to evaluate for tinea versicolor. An additional tool to aid in diagnosis is the use of a Wood’s light. The lesions of PMH characteristically show punctiform orange-red follicular fluorescence when exposed to a Wood’s light, indicating the presence of a porphyrin-producing organism, presumably P. acnes. A skin biopsy is necessary to rule out hypopigmented mycosis fungoides.
Skin biopsy of PMH typically reveals decreased melanin with a normal number of melanocytes. In our patient, a punch biopsy of the right lateral arm demonstrated minimally decreased density of epidermal melanocytes with dermal pigment incontinence. SOX10 immunohistochemical staining demonstrated scattered melanocytes in the epidermis. Preserved melanin within keratinocytes was noted.
In our patient, there was significant spread to the face, which is highly unusual and has only been documented in a few case series. There are no standard recommendations for definitive treatment of PMH. Topical antimicrobial therapies, such as clindamycin solution and benzoyl peroxide gel, have been beneficial in some studies. Tetracyclines, narrow-band ultraviolet B phototherapy, and even isotretinoin have had some reported success.
This case and photo were submitted by Mr. Franzetti, Dr. Rush, and Dr. Shalin of the University of Arkansas for Medical Sciences, Little Rock.
Donna Bilu Martin, MD, is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to dermnews@mdedge.com.
A healthy 8-year-old boy presents with several skin-colored, round 1-3 mm papules on the nose, forehead, and cheeks
A shave biopsy of one of the lesions was performed that showed a proliferation of nests of basaloid cells on the dermis with palisading and rare vacuolated clear cell change. A rare ductal structure with luminal proteinaceous contents was noted. The findings were consistent with a trichoepithelioma.
Trichoepitheliomas are rare, benign, adnexal skin tumors that can start in early childhood or during puberty. The lesions are most commonly seen in girls as skin color papules on the face, and sometimes on the trunk and the neck. Trichoepitheliomas can appear as a benign single lesion nonfamilial form or as a familial form with multiple lesions.1 Brooke-Spiegler syndrome (BSS) is a rare autosomal dominant condition where affected individuals have multiple trichoepitheliomas, cylindromas, and spiradenomas. Depending on the predominant type of lesion, phenotypic variants include multiple familial trichoepithelioma type 1 and familial cylindromatosis.2 BSS is caused by mutations within CYLD, a tumor-suppressor gene located on chromosome 16q12-q13.3 Our patient presented only with trichoepitheliomas with no other lesions on the scalp, neck, or torso.
Multiple trichoepitheliomas also can be seen in other syndromes including Rombo syndrome, which is characterized by basal cell carcinomas, milia, hypotrichosis, distal vasodilation, and atrophoderma vermiculata; none seen in our patient. Bazex-Dupré-Christol syndrome is an X-linked dominant condition in which affected individuals can present with multiple trichoepitheliomas, as well as milia, hypotrichosis, follicular atrophoderma, and basal cell carcinomas.
The differential diagnosis of skin color papules on the central face on a child should include acne, flat warts, and angiofibromas seen in tuberous sclerosis. Our patient’s lesions were monomorphous, and there were no comedones, pustules, or inflammatory papules characteristic of acne.
He had warts on his hands which could make it suspicious for the face lesions to be verrucous in nature. Flat warts also present as skin color papules, but characteristically are flat, not round and shiny as our patient’s lesions were. Angiofibromas, as seen in individuals with tuberous sclerosis, also can start at an early age in the same location as trichoepitheliomas in BSS, but clinically the lesions are pinker and redder rather than the skin-color, round shape papules characteristic of trichoepitheliomas. Patients may have other findings suggestive of tuberous sclerosis including confetti hypopigmentation, ash leaf spots, shagreen patch, and a history of seizures or developmental delay – none of which were present in our patient. Children with basal cell nevus syndrome can present with skin color to shiny telangiectatic papules (basal cell carcinomas) that can be single or multiple on the face, chest, and back. The lesions usually are not seen in clusters around the nose and central face as seen in patients with BSS. Patients with basal cell nevus syndrome can develop jaw bone cysts, brain tumors (medulloblastoma), and fibromas on the heart or ovaries, palmar pits and be macrocephalic.4
Trichoepitheliomas usually are treated surgically but other nonsurgical removing techniques include laser resurfacing, curettage, and electrocautery.5 Malignant transformation can occur in 5%-10% of the individuals and should be managed by a multidisciplinary team. Topical treatment with sirolimus previously has been reported to be effective in young patients.6
Dr. Matiz is a pediatric dermatologist at Southern California Permanente Medical Group, San Diego. She said she had no relevant financial disclosures. Email Dr. Matiz at pdnews@mdedge.com.
References
1. Acta Dermatovenerol Croat. 2018 Jun;26(2):162-5.
2. Eur J Med Genet. 2015;58(5):271-8.
3. Am J Dermatopathol. 2014;36(11):868-74.
4. Int J Dermatol. 2016 Apr;55(4):367-75.
5. Int J Dermatol. 2007;46(6):583-6.
6. Dermatol Ther. 2017 Mar. doi: 10.1111/dth.12458.
A shave biopsy of one of the lesions was performed that showed a proliferation of nests of basaloid cells on the dermis with palisading and rare vacuolated clear cell change. A rare ductal structure with luminal proteinaceous contents was noted. The findings were consistent with a trichoepithelioma.
Trichoepitheliomas are rare, benign, adnexal skin tumors that can start in early childhood or during puberty. The lesions are most commonly seen in girls as skin color papules on the face, and sometimes on the trunk and the neck. Trichoepitheliomas can appear as a benign single lesion nonfamilial form or as a familial form with multiple lesions.1 Brooke-Spiegler syndrome (BSS) is a rare autosomal dominant condition where affected individuals have multiple trichoepitheliomas, cylindromas, and spiradenomas. Depending on the predominant type of lesion, phenotypic variants include multiple familial trichoepithelioma type 1 and familial cylindromatosis.2 BSS is caused by mutations within CYLD, a tumor-suppressor gene located on chromosome 16q12-q13.3 Our patient presented only with trichoepitheliomas with no other lesions on the scalp, neck, or torso.
Multiple trichoepitheliomas also can be seen in other syndromes including Rombo syndrome, which is characterized by basal cell carcinomas, milia, hypotrichosis, distal vasodilation, and atrophoderma vermiculata; none seen in our patient. Bazex-Dupré-Christol syndrome is an X-linked dominant condition in which affected individuals can present with multiple trichoepitheliomas, as well as milia, hypotrichosis, follicular atrophoderma, and basal cell carcinomas.
The differential diagnosis of skin color papules on the central face on a child should include acne, flat warts, and angiofibromas seen in tuberous sclerosis. Our patient’s lesions were monomorphous, and there were no comedones, pustules, or inflammatory papules characteristic of acne.
He had warts on his hands which could make it suspicious for the face lesions to be verrucous in nature. Flat warts also present as skin color papules, but characteristically are flat, not round and shiny as our patient’s lesions were. Angiofibromas, as seen in individuals with tuberous sclerosis, also can start at an early age in the same location as trichoepitheliomas in BSS, but clinically the lesions are pinker and redder rather than the skin-color, round shape papules characteristic of trichoepitheliomas. Patients may have other findings suggestive of tuberous sclerosis including confetti hypopigmentation, ash leaf spots, shagreen patch, and a history of seizures or developmental delay – none of which were present in our patient. Children with basal cell nevus syndrome can present with skin color to shiny telangiectatic papules (basal cell carcinomas) that can be single or multiple on the face, chest, and back. The lesions usually are not seen in clusters around the nose and central face as seen in patients with BSS. Patients with basal cell nevus syndrome can develop jaw bone cysts, brain tumors (medulloblastoma), and fibromas on the heart or ovaries, palmar pits and be macrocephalic.4
Trichoepitheliomas usually are treated surgically but other nonsurgical removing techniques include laser resurfacing, curettage, and electrocautery.5 Malignant transformation can occur in 5%-10% of the individuals and should be managed by a multidisciplinary team. Topical treatment with sirolimus previously has been reported to be effective in young patients.6
Dr. Matiz is a pediatric dermatologist at Southern California Permanente Medical Group, San Diego. She said she had no relevant financial disclosures. Email Dr. Matiz at pdnews@mdedge.com.
References
1. Acta Dermatovenerol Croat. 2018 Jun;26(2):162-5.
2. Eur J Med Genet. 2015;58(5):271-8.
3. Am J Dermatopathol. 2014;36(11):868-74.
4. Int J Dermatol. 2016 Apr;55(4):367-75.
5. Int J Dermatol. 2007;46(6):583-6.
6. Dermatol Ther. 2017 Mar. doi: 10.1111/dth.12458.
A shave biopsy of one of the lesions was performed that showed a proliferation of nests of basaloid cells on the dermis with palisading and rare vacuolated clear cell change. A rare ductal structure with luminal proteinaceous contents was noted. The findings were consistent with a trichoepithelioma.
Trichoepitheliomas are rare, benign, adnexal skin tumors that can start in early childhood or during puberty. The lesions are most commonly seen in girls as skin color papules on the face, and sometimes on the trunk and the neck. Trichoepitheliomas can appear as a benign single lesion nonfamilial form or as a familial form with multiple lesions.1 Brooke-Spiegler syndrome (BSS) is a rare autosomal dominant condition where affected individuals have multiple trichoepitheliomas, cylindromas, and spiradenomas. Depending on the predominant type of lesion, phenotypic variants include multiple familial trichoepithelioma type 1 and familial cylindromatosis.2 BSS is caused by mutations within CYLD, a tumor-suppressor gene located on chromosome 16q12-q13.3 Our patient presented only with trichoepitheliomas with no other lesions on the scalp, neck, or torso.
Multiple trichoepitheliomas also can be seen in other syndromes including Rombo syndrome, which is characterized by basal cell carcinomas, milia, hypotrichosis, distal vasodilation, and atrophoderma vermiculata; none seen in our patient. Bazex-Dupré-Christol syndrome is an X-linked dominant condition in which affected individuals can present with multiple trichoepitheliomas, as well as milia, hypotrichosis, follicular atrophoderma, and basal cell carcinomas.
The differential diagnosis of skin color papules on the central face on a child should include acne, flat warts, and angiofibromas seen in tuberous sclerosis. Our patient’s lesions were monomorphous, and there were no comedones, pustules, or inflammatory papules characteristic of acne.
He had warts on his hands which could make it suspicious for the face lesions to be verrucous in nature. Flat warts also present as skin color papules, but characteristically are flat, not round and shiny as our patient’s lesions were. Angiofibromas, as seen in individuals with tuberous sclerosis, also can start at an early age in the same location as trichoepitheliomas in BSS, but clinically the lesions are pinker and redder rather than the skin-color, round shape papules characteristic of trichoepitheliomas. Patients may have other findings suggestive of tuberous sclerosis including confetti hypopigmentation, ash leaf spots, shagreen patch, and a history of seizures or developmental delay – none of which were present in our patient. Children with basal cell nevus syndrome can present with skin color to shiny telangiectatic papules (basal cell carcinomas) that can be single or multiple on the face, chest, and back. The lesions usually are not seen in clusters around the nose and central face as seen in patients with BSS. Patients with basal cell nevus syndrome can develop jaw bone cysts, brain tumors (medulloblastoma), and fibromas on the heart or ovaries, palmar pits and be macrocephalic.4
Trichoepitheliomas usually are treated surgically but other nonsurgical removing techniques include laser resurfacing, curettage, and electrocautery.5 Malignant transformation can occur in 5%-10% of the individuals and should be managed by a multidisciplinary team. Topical treatment with sirolimus previously has been reported to be effective in young patients.6
Dr. Matiz is a pediatric dermatologist at Southern California Permanente Medical Group, San Diego. She said she had no relevant financial disclosures. Email Dr. Matiz at pdnews@mdedge.com.
References
1. Acta Dermatovenerol Croat. 2018 Jun;26(2):162-5.
2. Eur J Med Genet. 2015;58(5):271-8.
3. Am J Dermatopathol. 2014;36(11):868-74.
4. Int J Dermatol. 2016 Apr;55(4):367-75.
5. Int J Dermatol. 2007;46(6):583-6.
6. Dermatol Ther. 2017 Mar. doi: 10.1111/dth.12458.
A white 8-year-old boy comes to our pediatric dermatology clinic with his mother for evaluation of acne. The lesions started about a year ago on his nose and now have spread to his cheeks. The bumps are not symptomatic. He has been applying over the counter salicylic acid and benzoyl peroxide gels with no help. The mother reports he has been growing well, denies any growth spurt, no axillary or genital hair or body odor noted.
None of the family members have a history of acne. The mother cannot recall any family members with similar lesions on the face. He has had some warts on his fingers for years and has been treated with over the counter salicylic acid. There is no family history of skin cancer.
On physical exam, he is a healthy young boy with several skin color, round papules 1-3 mm on the nose, forehead, and cheeks. There are no lesions on the scalp. He has abundant brown hair. He has few verrucous papules on the fingers. Axillary and genital hair is not noted. There is no body odor and he is Tanner stage I.
An 89-year-old woman presented with an ulceration overlying a cardiac pacemaker
Cardiac implantable electronic devices (CIEDs) – cardiac pacemakers and implantable cardioverter defibrillators –are an established treatment for the management of cardiac dysrhythmias in millions of patients. Complications occur in up to 15%, some of which may present first to the dermatologist.
The differential (caused by local venous obstruction and pressure dermatitis), and impending skin erosion/device extrusion.
Erosion and extrusion is a major complication with significant morbidity and mortality. The two main causes are pressure necrosis and infection. Pressure necrosis is influenced by the size of the device, complexity of the connections, and technical skill with which the pacemaker chest wall pocket is created.
After extrusion, the pacemaker should be considered contaminated and removed, and the necrotic tissue debrided. If infected, a prolonged course of appropriate antibiotic therapy is indicated. A bacterial culture in the patient presented here was negative.
Pocket infection of CIEDs is rare and may manifest as erythema, tenderness, drainage, erosion, or pruritus above the site of the pacemaker, along with systemic symptoms and signs, including fever, chills, or malaise. Some may have just the systemic symptoms. Fewer than half of patients with CIED infection present within 1 year of their last procedure.
Ruptured epidermal cysts usually manifest as acute swelling, inflammation, and tenderness of previously long-standing asymptomatic epidermal cysts. There may be drainage of malodorous keratinous and purulent debris. They are typically not infected. Treatment includes incision and drainage for fluctuant lesions or intralesional corticosteroid injection for early, nonfluctuant cases.
Allergic contact dermatitis to metal may be seen with implantable devices. Patch testing to various metal allergens can be helpful in determining if any allergy is present.
This case and photo were submitted by Michael Stierstorfer, MD, East Penn Dermatology, North Wales, Pa.
Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to dermnews@mdedge.com.
Cardiac implantable electronic devices (CIEDs) – cardiac pacemakers and implantable cardioverter defibrillators –are an established treatment for the management of cardiac dysrhythmias in millions of patients. Complications occur in up to 15%, some of which may present first to the dermatologist.
The differential (caused by local venous obstruction and pressure dermatitis), and impending skin erosion/device extrusion.
Erosion and extrusion is a major complication with significant morbidity and mortality. The two main causes are pressure necrosis and infection. Pressure necrosis is influenced by the size of the device, complexity of the connections, and technical skill with which the pacemaker chest wall pocket is created.
After extrusion, the pacemaker should be considered contaminated and removed, and the necrotic tissue debrided. If infected, a prolonged course of appropriate antibiotic therapy is indicated. A bacterial culture in the patient presented here was negative.
Pocket infection of CIEDs is rare and may manifest as erythema, tenderness, drainage, erosion, or pruritus above the site of the pacemaker, along with systemic symptoms and signs, including fever, chills, or malaise. Some may have just the systemic symptoms. Fewer than half of patients with CIED infection present within 1 year of their last procedure.
Ruptured epidermal cysts usually manifest as acute swelling, inflammation, and tenderness of previously long-standing asymptomatic epidermal cysts. There may be drainage of malodorous keratinous and purulent debris. They are typically not infected. Treatment includes incision and drainage for fluctuant lesions or intralesional corticosteroid injection for early, nonfluctuant cases.
Allergic contact dermatitis to metal may be seen with implantable devices. Patch testing to various metal allergens can be helpful in determining if any allergy is present.
This case and photo were submitted by Michael Stierstorfer, MD, East Penn Dermatology, North Wales, Pa.
Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to dermnews@mdedge.com.
Cardiac implantable electronic devices (CIEDs) – cardiac pacemakers and implantable cardioverter defibrillators –are an established treatment for the management of cardiac dysrhythmias in millions of patients. Complications occur in up to 15%, some of which may present first to the dermatologist.
The differential (caused by local venous obstruction and pressure dermatitis), and impending skin erosion/device extrusion.
Erosion and extrusion is a major complication with significant morbidity and mortality. The two main causes are pressure necrosis and infection. Pressure necrosis is influenced by the size of the device, complexity of the connections, and technical skill with which the pacemaker chest wall pocket is created.
After extrusion, the pacemaker should be considered contaminated and removed, and the necrotic tissue debrided. If infected, a prolonged course of appropriate antibiotic therapy is indicated. A bacterial culture in the patient presented here was negative.
Pocket infection of CIEDs is rare and may manifest as erythema, tenderness, drainage, erosion, or pruritus above the site of the pacemaker, along with systemic symptoms and signs, including fever, chills, or malaise. Some may have just the systemic symptoms. Fewer than half of patients with CIED infection present within 1 year of their last procedure.
Ruptured epidermal cysts usually manifest as acute swelling, inflammation, and tenderness of previously long-standing asymptomatic epidermal cysts. There may be drainage of malodorous keratinous and purulent debris. They are typically not infected. Treatment includes incision and drainage for fluctuant lesions or intralesional corticosteroid injection for early, nonfluctuant cases.
Allergic contact dermatitis to metal may be seen with implantable devices. Patch testing to various metal allergens can be helpful in determining if any allergy is present.
This case and photo were submitted by Michael Stierstorfer, MD, East Penn Dermatology, North Wales, Pa.
Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to dermnews@mdedge.com.
A 5-year-old boy with a papular rash on his arm
Lichen striatus (LS) is a common benign skin condition that presents in children between the ages of 5 and 15 years.1 The rash is typically unilateral and most frequently on the extremities, although it may appear on the face, trunk, or buttocks. The lesions start as pink or skin-colored asymptomatic papules in a linear orientation following the lines of Blaschko. There may be residual postinflammatory hypo- or hyperpigmentation which often improves within a few years.
Of note, there are subsets of lichen striatus: Hypopigmented lichen striatus with minimal papules has been termed “lichen striatus albus.” Nail lichen striatus may present as onycholysis or fissuring of nails, present as an isolated finding, or more commonly in association with concurrent affected skin. Nail lichen striatus typically resolves on its own, however there are case reports of improvement with intralesional steroids.2
There is no established etiology for LS. Autoimmune disease, viruses, immunizations, medications, and hypersensitivity reactions have been associated with triggering LS in various case reports, although strength of the associations is low. Children have been reported to have LS following scarlet fever and Candida vulvitis.3 Diagnosis usually is clinical, although biopsy may be helpful for histopathologic confirmation. No work-up for associated infections or conditions is warranted.
The differential for linear papular lesions includes inflammatory linear verrucous epidermal nevus (ILVEN), blaschkitis, or linear morphea. ILVEN is a hamartoma that usually is congenital or presents in early childhood; presents with linear or whorled, hyperkeratotic papules and plaque in similar linear “line of Blaschko” patterns; and represents cutaneous mosaicism. It is often difficult to differentiate between lichen striatus and ILVEN, however lichen striatus is not congenital, and is a self-limited condition. Under dermoscopy (polarized light systems) findings of LS more frequently demonstrate gray granular pigmentation. ILVEN is more frequently associated with cerebriform pattern.4 Blaschkitis is a term for a blaschkoid inflammation of the skin that presents with more eczematous findings and histology of spongiosis, unlike the lichenoid findings of LS. It is typically accompanied by noticeable pruritus and broader bands of involved area, and has older age of onset than LS. Linear morphea is a deeper inflammatory process of the dermis or subcutaneous fat, presenting with sclerotic skin, and typically has associated atrophy.
Treatment need not be pursued for lichen striatus because it is a benign condition. The lesions typically self-resolve without any residual scarring. If patients have associated pruritus then low- to midpotency topical steroids can be used for symptomatic relief.
Dr. Kaushik is with the division of pediatric and adolescent dermatology at Rady Children’s Hospital-San Diego, and Dr. Eichenfield is chief of pediatric and adolescent dermatology at Rady Children’s Hospital-San Diego. He is vice chair of the department of dermatology and professor of dermatology and pediatrics at the University of California, San Diego. There are no conflicts of interest or financial disclosures for Dr. Kaushik or Dr. Eichenfield. Email them at pdnews@mdedge.com.
References
1. Gupta D, Mathes E. Lichen Striatus. (Levy ML ed.) 2019: UpToDate.
2. Dermatol Ther. 2018 Nov;31(6):e12713.
3. Int J Dermatol. 2018 Sep;57(9):1118-9.
4. J Dermatol. 2017 Dec;44(12):e355-6.
Lichen striatus (LS) is a common benign skin condition that presents in children between the ages of 5 and 15 years.1 The rash is typically unilateral and most frequently on the extremities, although it may appear on the face, trunk, or buttocks. The lesions start as pink or skin-colored asymptomatic papules in a linear orientation following the lines of Blaschko. There may be residual postinflammatory hypo- or hyperpigmentation which often improves within a few years.
Of note, there are subsets of lichen striatus: Hypopigmented lichen striatus with minimal papules has been termed “lichen striatus albus.” Nail lichen striatus may present as onycholysis or fissuring of nails, present as an isolated finding, or more commonly in association with concurrent affected skin. Nail lichen striatus typically resolves on its own, however there are case reports of improvement with intralesional steroids.2
There is no established etiology for LS. Autoimmune disease, viruses, immunizations, medications, and hypersensitivity reactions have been associated with triggering LS in various case reports, although strength of the associations is low. Children have been reported to have LS following scarlet fever and Candida vulvitis.3 Diagnosis usually is clinical, although biopsy may be helpful for histopathologic confirmation. No work-up for associated infections or conditions is warranted.
The differential for linear papular lesions includes inflammatory linear verrucous epidermal nevus (ILVEN), blaschkitis, or linear morphea. ILVEN is a hamartoma that usually is congenital or presents in early childhood; presents with linear or whorled, hyperkeratotic papules and plaque in similar linear “line of Blaschko” patterns; and represents cutaneous mosaicism. It is often difficult to differentiate between lichen striatus and ILVEN, however lichen striatus is not congenital, and is a self-limited condition. Under dermoscopy (polarized light systems) findings of LS more frequently demonstrate gray granular pigmentation. ILVEN is more frequently associated with cerebriform pattern.4 Blaschkitis is a term for a blaschkoid inflammation of the skin that presents with more eczematous findings and histology of spongiosis, unlike the lichenoid findings of LS. It is typically accompanied by noticeable pruritus and broader bands of involved area, and has older age of onset than LS. Linear morphea is a deeper inflammatory process of the dermis or subcutaneous fat, presenting with sclerotic skin, and typically has associated atrophy.
Treatment need not be pursued for lichen striatus because it is a benign condition. The lesions typically self-resolve without any residual scarring. If patients have associated pruritus then low- to midpotency topical steroids can be used for symptomatic relief.
Dr. Kaushik is with the division of pediatric and adolescent dermatology at Rady Children’s Hospital-San Diego, and Dr. Eichenfield is chief of pediatric and adolescent dermatology at Rady Children’s Hospital-San Diego. He is vice chair of the department of dermatology and professor of dermatology and pediatrics at the University of California, San Diego. There are no conflicts of interest or financial disclosures for Dr. Kaushik or Dr. Eichenfield. Email them at pdnews@mdedge.com.
References
1. Gupta D, Mathes E. Lichen Striatus. (Levy ML ed.) 2019: UpToDate.
2. Dermatol Ther. 2018 Nov;31(6):e12713.
3. Int J Dermatol. 2018 Sep;57(9):1118-9.
4. J Dermatol. 2017 Dec;44(12):e355-6.
Lichen striatus (LS) is a common benign skin condition that presents in children between the ages of 5 and 15 years.1 The rash is typically unilateral and most frequently on the extremities, although it may appear on the face, trunk, or buttocks. The lesions start as pink or skin-colored asymptomatic papules in a linear orientation following the lines of Blaschko. There may be residual postinflammatory hypo- or hyperpigmentation which often improves within a few years.
Of note, there are subsets of lichen striatus: Hypopigmented lichen striatus with minimal papules has been termed “lichen striatus albus.” Nail lichen striatus may present as onycholysis or fissuring of nails, present as an isolated finding, or more commonly in association with concurrent affected skin. Nail lichen striatus typically resolves on its own, however there are case reports of improvement with intralesional steroids.2
There is no established etiology for LS. Autoimmune disease, viruses, immunizations, medications, and hypersensitivity reactions have been associated with triggering LS in various case reports, although strength of the associations is low. Children have been reported to have LS following scarlet fever and Candida vulvitis.3 Diagnosis usually is clinical, although biopsy may be helpful for histopathologic confirmation. No work-up for associated infections or conditions is warranted.
The differential for linear papular lesions includes inflammatory linear verrucous epidermal nevus (ILVEN), blaschkitis, or linear morphea. ILVEN is a hamartoma that usually is congenital or presents in early childhood; presents with linear or whorled, hyperkeratotic papules and plaque in similar linear “line of Blaschko” patterns; and represents cutaneous mosaicism. It is often difficult to differentiate between lichen striatus and ILVEN, however lichen striatus is not congenital, and is a self-limited condition. Under dermoscopy (polarized light systems) findings of LS more frequently demonstrate gray granular pigmentation. ILVEN is more frequently associated with cerebriform pattern.4 Blaschkitis is a term for a blaschkoid inflammation of the skin that presents with more eczematous findings and histology of spongiosis, unlike the lichenoid findings of LS. It is typically accompanied by noticeable pruritus and broader bands of involved area, and has older age of onset than LS. Linear morphea is a deeper inflammatory process of the dermis or subcutaneous fat, presenting with sclerotic skin, and typically has associated atrophy.
Treatment need not be pursued for lichen striatus because it is a benign condition. The lesions typically self-resolve without any residual scarring. If patients have associated pruritus then low- to midpotency topical steroids can be used for symptomatic relief.
Dr. Kaushik is with the division of pediatric and adolescent dermatology at Rady Children’s Hospital-San Diego, and Dr. Eichenfield is chief of pediatric and adolescent dermatology at Rady Children’s Hospital-San Diego. He is vice chair of the department of dermatology and professor of dermatology and pediatrics at the University of California, San Diego. There are no conflicts of interest or financial disclosures for Dr. Kaushik or Dr. Eichenfield. Email them at pdnews@mdedge.com.
References
1. Gupta D, Mathes E. Lichen Striatus. (Levy ML ed.) 2019: UpToDate.
2. Dermatol Ther. 2018 Nov;31(6):e12713.
3. Int J Dermatol. 2018 Sep;57(9):1118-9.
4. J Dermatol. 2017 Dec;44(12):e355-6.
A 72-year-old white male with a history of psoriatic arthritis presented with a 1-year history of multiple, intermittently pruritic papules on his face and trunk
Scleromyxedema
area, characteristically involving the glabella and ears. In some patients, the skin may be intensely pruritic, but this is not a universal finding and varies considerably among patients. In addition to affecting the skin, scleromyxedema has variable multisystem effects on the gastrointestinal tract, and musculoskeletal, pulmonary, cardiovascular, renal, and central nervous systems. The most common symptoms are proximal muscle weakness, dysphagia, and dyspnea on exertion. Scleromyxedema can also be associated with a paraproteinemia, mainly immunoglobulin G-lambda type.
Scleromyxedema shares some features with other cutaneous diseases, and the main differential diagnosis includes localized scleromyxedema, also known as lichen myxedematosus. Lichen myxedematosus presents with waxy, firm papules and plaques. Systemic involvement and monoclonal gammopathy are characteristically absent. Scleroderma differs given the increase in fibrosis of cutaneous lesions, a higher percentage of Raynaud’s phenomenon, prominent lung disease, and autoantibodies.
On histopathologic review, scleromyxedema is associated with papular and mucin deposition, and increased fibroblast proliferation. The punch biopsy of the exhibited patient demonstrated a dome-shaped dermal nodule composed of fibroblasts in an edematous stroma. An Alcian blue stain highlighted increased mucin in the dermis and S-100 staining highlighted rare cells in the dermis.
The treatment of choice for scleromyxedema varies but includes intravenous immunoglobulins, systemic glucocorticosteroids, thalidomide, or immunosuppressant medications. In this patient, an IgG paraproteinemia was found on serum protein electrophoresis. The patient was evaluated by hematology-oncology, and no underlying myeloproliferative or dysplastic disease was found. The patient was started on intravenous immunoglobulin infusions with near complete resolution of his eruption and arthritic symptoms.
This case and photo were submitted by Jennifer Maldonado, a medical student at Nova Southeastern University, Ft. Lauderdale, Fla., and Kate Oberlin, MD, and Brian Morrison, MD, of the department of dermatology and cutaneous surgery, University of Miami; and Michelle Demory Beckler, PhD, of the College of Osteopathic Medicine and the department of microbiology, College of Medical Sciences, Nova Southeastern University.
Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to dermnews@mdedge.com.
Scleromyxedema
area, characteristically involving the glabella and ears. In some patients, the skin may be intensely pruritic, but this is not a universal finding and varies considerably among patients. In addition to affecting the skin, scleromyxedema has variable multisystem effects on the gastrointestinal tract, and musculoskeletal, pulmonary, cardiovascular, renal, and central nervous systems. The most common symptoms are proximal muscle weakness, dysphagia, and dyspnea on exertion. Scleromyxedema can also be associated with a paraproteinemia, mainly immunoglobulin G-lambda type.
Scleromyxedema shares some features with other cutaneous diseases, and the main differential diagnosis includes localized scleromyxedema, also known as lichen myxedematosus. Lichen myxedematosus presents with waxy, firm papules and plaques. Systemic involvement and monoclonal gammopathy are characteristically absent. Scleroderma differs given the increase in fibrosis of cutaneous lesions, a higher percentage of Raynaud’s phenomenon, prominent lung disease, and autoantibodies.
On histopathologic review, scleromyxedema is associated with papular and mucin deposition, and increased fibroblast proliferation. The punch biopsy of the exhibited patient demonstrated a dome-shaped dermal nodule composed of fibroblasts in an edematous stroma. An Alcian blue stain highlighted increased mucin in the dermis and S-100 staining highlighted rare cells in the dermis.
The treatment of choice for scleromyxedema varies but includes intravenous immunoglobulins, systemic glucocorticosteroids, thalidomide, or immunosuppressant medications. In this patient, an IgG paraproteinemia was found on serum protein electrophoresis. The patient was evaluated by hematology-oncology, and no underlying myeloproliferative or dysplastic disease was found. The patient was started on intravenous immunoglobulin infusions with near complete resolution of his eruption and arthritic symptoms.
This case and photo were submitted by Jennifer Maldonado, a medical student at Nova Southeastern University, Ft. Lauderdale, Fla., and Kate Oberlin, MD, and Brian Morrison, MD, of the department of dermatology and cutaneous surgery, University of Miami; and Michelle Demory Beckler, PhD, of the College of Osteopathic Medicine and the department of microbiology, College of Medical Sciences, Nova Southeastern University.
Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to dermnews@mdedge.com.
Scleromyxedema
area, characteristically involving the glabella and ears. In some patients, the skin may be intensely pruritic, but this is not a universal finding and varies considerably among patients. In addition to affecting the skin, scleromyxedema has variable multisystem effects on the gastrointestinal tract, and musculoskeletal, pulmonary, cardiovascular, renal, and central nervous systems. The most common symptoms are proximal muscle weakness, dysphagia, and dyspnea on exertion. Scleromyxedema can also be associated with a paraproteinemia, mainly immunoglobulin G-lambda type.
Scleromyxedema shares some features with other cutaneous diseases, and the main differential diagnosis includes localized scleromyxedema, also known as lichen myxedematosus. Lichen myxedematosus presents with waxy, firm papules and plaques. Systemic involvement and monoclonal gammopathy are characteristically absent. Scleroderma differs given the increase in fibrosis of cutaneous lesions, a higher percentage of Raynaud’s phenomenon, prominent lung disease, and autoantibodies.
On histopathologic review, scleromyxedema is associated with papular and mucin deposition, and increased fibroblast proliferation. The punch biopsy of the exhibited patient demonstrated a dome-shaped dermal nodule composed of fibroblasts in an edematous stroma. An Alcian blue stain highlighted increased mucin in the dermis and S-100 staining highlighted rare cells in the dermis.
The treatment of choice for scleromyxedema varies but includes intravenous immunoglobulins, systemic glucocorticosteroids, thalidomide, or immunosuppressant medications. In this patient, an IgG paraproteinemia was found on serum protein electrophoresis. The patient was evaluated by hematology-oncology, and no underlying myeloproliferative or dysplastic disease was found. The patient was started on intravenous immunoglobulin infusions with near complete resolution of his eruption and arthritic symptoms.
This case and photo were submitted by Jennifer Maldonado, a medical student at Nova Southeastern University, Ft. Lauderdale, Fla., and Kate Oberlin, MD, and Brian Morrison, MD, of the department of dermatology and cutaneous surgery, University of Miami; and Michelle Demory Beckler, PhD, of the College of Osteopathic Medicine and the department of microbiology, College of Medical Sciences, Nova Southeastern University.
Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to dermnews@mdedge.com.
A 60-year-old white male presented with a painful nodule on the right lateral thigh that had been present for years
Benign tumors consisting of glomus cells may be subdivided into two types: glomus tumors and glomuvenous malformations or glomangiomas. Glomus cells are modified smooth muscle cells that normally line the Sucquet-Hoyer canal, an arteriovenous fistula that is involved in temperature regulation in the digits.
. In women, lesions more frequently occur on the fingers (especially nail beds). Glomus tumors are firm subcutaneous nodules, often skin colored or bluish in color. Subungual tumors tend to appear bluish under the nail plate. Lesions are extremely tender or painful, with worse pain upon palpation. Occasionally, nontender lesions can be seen.
In children, multiple nontender lesions are called glomangiomas or glomuvenous malformations. They may be sporadic or can be inherited in an autosomal dominant fashion due to a mutation in glomulin on chromosome 1p21-p22. Multiple lesions may be scattered or grouped, often in a segmental distribution. Congenital lesions tend to be large, blue-purple in color with a cobblestone appearance. They are more superficial than venous malformations.
Histologically, a proliferation of blood vessels surrounded by glomus cells is seen. Glomus cells appear as monotonous cells with a dense, round nucleus and abundant pink cytoplasm. Glomus cells can also be appreciated single-filing through pale stroma, resembling strings of black pearls. Glomus cells stain positive for smooth muscle actin and vimentin.
The painful tumor differential diagnosis has been described in the literature by the mnemonic “LEND AN EGG:” leiomyoma, eccrine spiradenoma, neuroma, dermatofibroma, angiolipoma, neurilemmoma, endometrioma, glomus tumor, and granular cell tumor.
The malignant counterpart is glomangiosarcoma, which is a rare tumor. These lesions are often large and deeply located on the extremities. Histologically, sarcomatous areas are mixed with areas of benign glomus tumor.
Surgical excision is the treatment of choice for solitary glomus tumors to provide pain relief. Subungual tumors are more challenging due to their small size, but may be excised as well. Glomuvenous malformations may require different treatment modalities, such as surgery and laser, due to their larger size.
This case and photo were submitted by Dr. Bilu Martin.
Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at www.mdedge.com/dermatology. To submit a case for possible publication, send an email to dermnews@mdedge.com.
Benign tumors consisting of glomus cells may be subdivided into two types: glomus tumors and glomuvenous malformations or glomangiomas. Glomus cells are modified smooth muscle cells that normally line the Sucquet-Hoyer canal, an arteriovenous fistula that is involved in temperature regulation in the digits.
. In women, lesions more frequently occur on the fingers (especially nail beds). Glomus tumors are firm subcutaneous nodules, often skin colored or bluish in color. Subungual tumors tend to appear bluish under the nail plate. Lesions are extremely tender or painful, with worse pain upon palpation. Occasionally, nontender lesions can be seen.
In children, multiple nontender lesions are called glomangiomas or glomuvenous malformations. They may be sporadic or can be inherited in an autosomal dominant fashion due to a mutation in glomulin on chromosome 1p21-p22. Multiple lesions may be scattered or grouped, often in a segmental distribution. Congenital lesions tend to be large, blue-purple in color with a cobblestone appearance. They are more superficial than venous malformations.
Histologically, a proliferation of blood vessels surrounded by glomus cells is seen. Glomus cells appear as monotonous cells with a dense, round nucleus and abundant pink cytoplasm. Glomus cells can also be appreciated single-filing through pale stroma, resembling strings of black pearls. Glomus cells stain positive for smooth muscle actin and vimentin.
The painful tumor differential diagnosis has been described in the literature by the mnemonic “LEND AN EGG:” leiomyoma, eccrine spiradenoma, neuroma, dermatofibroma, angiolipoma, neurilemmoma, endometrioma, glomus tumor, and granular cell tumor.
The malignant counterpart is glomangiosarcoma, which is a rare tumor. These lesions are often large and deeply located on the extremities. Histologically, sarcomatous areas are mixed with areas of benign glomus tumor.
Surgical excision is the treatment of choice for solitary glomus tumors to provide pain relief. Subungual tumors are more challenging due to their small size, but may be excised as well. Glomuvenous malformations may require different treatment modalities, such as surgery and laser, due to their larger size.
This case and photo were submitted by Dr. Bilu Martin.
Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at www.mdedge.com/dermatology. To submit a case for possible publication, send an email to dermnews@mdedge.com.
Benign tumors consisting of glomus cells may be subdivided into two types: glomus tumors and glomuvenous malformations or glomangiomas. Glomus cells are modified smooth muscle cells that normally line the Sucquet-Hoyer canal, an arteriovenous fistula that is involved in temperature regulation in the digits.
. In women, lesions more frequently occur on the fingers (especially nail beds). Glomus tumors are firm subcutaneous nodules, often skin colored or bluish in color. Subungual tumors tend to appear bluish under the nail plate. Lesions are extremely tender or painful, with worse pain upon palpation. Occasionally, nontender lesions can be seen.
In children, multiple nontender lesions are called glomangiomas or glomuvenous malformations. They may be sporadic or can be inherited in an autosomal dominant fashion due to a mutation in glomulin on chromosome 1p21-p22. Multiple lesions may be scattered or grouped, often in a segmental distribution. Congenital lesions tend to be large, blue-purple in color with a cobblestone appearance. They are more superficial than venous malformations.
Histologically, a proliferation of blood vessels surrounded by glomus cells is seen. Glomus cells appear as monotonous cells with a dense, round nucleus and abundant pink cytoplasm. Glomus cells can also be appreciated single-filing through pale stroma, resembling strings of black pearls. Glomus cells stain positive for smooth muscle actin and vimentin.
The painful tumor differential diagnosis has been described in the literature by the mnemonic “LEND AN EGG:” leiomyoma, eccrine spiradenoma, neuroma, dermatofibroma, angiolipoma, neurilemmoma, endometrioma, glomus tumor, and granular cell tumor.
The malignant counterpart is glomangiosarcoma, which is a rare tumor. These lesions are often large and deeply located on the extremities. Histologically, sarcomatous areas are mixed with areas of benign glomus tumor.
Surgical excision is the treatment of choice for solitary glomus tumors to provide pain relief. Subungual tumors are more challenging due to their small size, but may be excised as well. Glomuvenous malformations may require different treatment modalities, such as surgery and laser, due to their larger size.
This case and photo were submitted by Dr. Bilu Martin.
Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at www.mdedge.com/dermatology. To submit a case for possible publication, send an email to dermnews@mdedge.com.
A 60-year-old white male presented with a painful nodule on the right lateral thigh that had been present for years. It has slowly been increasing in size over time.
A 13-month-old, healthy black male presented with a 6-month history of dry, scaly skin on the body
Ichthyosis vulgaris
Ichthyoses describe a group of disorders of cornification in which the epidermis differentiates abnormally, leading to generalized scaling of the skin. Ichthyosis is derived from the Greek word for fish, “ichthys.” Ichthyosis vulgaris is the most common of these conditions and often presents in early childhood during the first year of life. It is inherited in an autosomal-dominant pattern. Skin is dry and scaly over the entire body, although the antecubital and popliteal fossa may be uninvolved. The scalp may be involved as well. Atopy and keratosis pilaris may be associated. By adulthood, symptoms tend to abate.
X-linked ichthyosis is an X-linked recessive trait, in which males are affected and mothers are carriers. The condition is caused by a deficiency of steroid sulfatase. This deficiency can result in low levels of estrogen during pregnancy in the mother of an affected fetus, hampering labor progression, and often requiring C-section. Children usually present before 3 months of age. Scales are large and dark. The antecubital and popliteal fossa are usually spared. The neck almost always is involved, coining the term “dirty neck disease.” Corneal opacities are present upon ophthalmologic examination. There is an increased risk of cryptorchidism and testicular cancer. Skin symptoms tend to worsen into adulthood.
Lamellar ichthyosis generally occurs at birth with a striking collodion-type membrane covering the body and underlying erythroderma, which then desquamates. Ectropion is usually present as well. Resulting scales are large and gray-brown. Lamellar ichthyosis is inherited in an autosomal recessive pattern. Mutations in transglutaminase 1 (TGM1), ALOXE3, ALOX12B, and ABCA12 genes have been implicated in this disorder.
Acquired ichthyosis can appear clinically similar to ichthyosis vulgaris. It occurs in patients with systemic diseases such as Hodgkin disease, non-Hodgkin lymphoma, mycosis fungoides, multiple myeloma, hypothyroidism, sarcoidosis, AIDS, and others.
improve hyperkeratosis. Urea-containing products can be helpful. Salicylic acid may be used but merit caution in children because of salicylate toxicity. Oral and topical retinoid can be helpful in lamellar ichthyosis.
This case and photo were submitted by Dr. Bilu Martin.
Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/edermatologynews.com. To submit a case for possible publication, send an email to dermnews@mdedge.com.
Ichthyosis vulgaris
Ichthyoses describe a group of disorders of cornification in which the epidermis differentiates abnormally, leading to generalized scaling of the skin. Ichthyosis is derived from the Greek word for fish, “ichthys.” Ichthyosis vulgaris is the most common of these conditions and often presents in early childhood during the first year of life. It is inherited in an autosomal-dominant pattern. Skin is dry and scaly over the entire body, although the antecubital and popliteal fossa may be uninvolved. The scalp may be involved as well. Atopy and keratosis pilaris may be associated. By adulthood, symptoms tend to abate.
X-linked ichthyosis is an X-linked recessive trait, in which males are affected and mothers are carriers. The condition is caused by a deficiency of steroid sulfatase. This deficiency can result in low levels of estrogen during pregnancy in the mother of an affected fetus, hampering labor progression, and often requiring C-section. Children usually present before 3 months of age. Scales are large and dark. The antecubital and popliteal fossa are usually spared. The neck almost always is involved, coining the term “dirty neck disease.” Corneal opacities are present upon ophthalmologic examination. There is an increased risk of cryptorchidism and testicular cancer. Skin symptoms tend to worsen into adulthood.
Lamellar ichthyosis generally occurs at birth with a striking collodion-type membrane covering the body and underlying erythroderma, which then desquamates. Ectropion is usually present as well. Resulting scales are large and gray-brown. Lamellar ichthyosis is inherited in an autosomal recessive pattern. Mutations in transglutaminase 1 (TGM1), ALOXE3, ALOX12B, and ABCA12 genes have been implicated in this disorder.
Acquired ichthyosis can appear clinically similar to ichthyosis vulgaris. It occurs in patients with systemic diseases such as Hodgkin disease, non-Hodgkin lymphoma, mycosis fungoides, multiple myeloma, hypothyroidism, sarcoidosis, AIDS, and others.
improve hyperkeratosis. Urea-containing products can be helpful. Salicylic acid may be used but merit caution in children because of salicylate toxicity. Oral and topical retinoid can be helpful in lamellar ichthyosis.
This case and photo were submitted by Dr. Bilu Martin.
Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/edermatologynews.com. To submit a case for possible publication, send an email to dermnews@mdedge.com.
Ichthyosis vulgaris
Ichthyoses describe a group of disorders of cornification in which the epidermis differentiates abnormally, leading to generalized scaling of the skin. Ichthyosis is derived from the Greek word for fish, “ichthys.” Ichthyosis vulgaris is the most common of these conditions and often presents in early childhood during the first year of life. It is inherited in an autosomal-dominant pattern. Skin is dry and scaly over the entire body, although the antecubital and popliteal fossa may be uninvolved. The scalp may be involved as well. Atopy and keratosis pilaris may be associated. By adulthood, symptoms tend to abate.
X-linked ichthyosis is an X-linked recessive trait, in which males are affected and mothers are carriers. The condition is caused by a deficiency of steroid sulfatase. This deficiency can result in low levels of estrogen during pregnancy in the mother of an affected fetus, hampering labor progression, and often requiring C-section. Children usually present before 3 months of age. Scales are large and dark. The antecubital and popliteal fossa are usually spared. The neck almost always is involved, coining the term “dirty neck disease.” Corneal opacities are present upon ophthalmologic examination. There is an increased risk of cryptorchidism and testicular cancer. Skin symptoms tend to worsen into adulthood.
Lamellar ichthyosis generally occurs at birth with a striking collodion-type membrane covering the body and underlying erythroderma, which then desquamates. Ectropion is usually present as well. Resulting scales are large and gray-brown. Lamellar ichthyosis is inherited in an autosomal recessive pattern. Mutations in transglutaminase 1 (TGM1), ALOXE3, ALOX12B, and ABCA12 genes have been implicated in this disorder.
Acquired ichthyosis can appear clinically similar to ichthyosis vulgaris. It occurs in patients with systemic diseases such as Hodgkin disease, non-Hodgkin lymphoma, mycosis fungoides, multiple myeloma, hypothyroidism, sarcoidosis, AIDS, and others.
improve hyperkeratosis. Urea-containing products can be helpful. Salicylic acid may be used but merit caution in children because of salicylate toxicity. Oral and topical retinoid can be helpful in lamellar ichthyosis.
This case and photo were submitted by Dr. Bilu Martin.
Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/edermatologynews.com. To submit a case for possible publication, send an email to dermnews@mdedge.com.
A 13-month-old, healthy black male presented with a 6-month history of dry, scaly skin on the body, including scalp and extremities. His neck was unaffected. His mother reports an uneventful pregnancy and natural childbirth. He had been prescribed triamcinolone in the past for eczema.
A 60-year-old white woman presented with a 3-month history of a painful, nonhealing ulceration on her left lateral lower leg
It is a vasculopathy rather than a vasculitis as the former is caused by occlusion of blood vessels and the latter results from inflammation of the vessels. Middle-aged women tend to be affected more frequently. Although the exact cause is unclear, systemic diseases, such as hypercoagulable states, may predispose vessels to develop occlusion. Associated disorders include antiphospholipid syndrome, protein C deficiency, factor V mutation, arteriosclerosis, hyperhomocysteinemia, and hepatitis C.
Typically, lesions begin as painful purpura or reticulated macules on the lower extremities that ulcerate and heal very slowly. Ankles, particularly malleoli, are more frequently affected. When they heal, they form painless white stellate scars typical of atrophie blanche. Surrounding erythema, telangiectasias, and sclerosis may be present; livedo reticularis may be seen as well.
Histologically, the epidermis may be atrophic or necrotic. Hyaline thickening of the blood vessel walls is seen. Thrombi may be present. Direct immunofluorescence of perilesional skin may be positive for complement C3 and immunoglobulin (IgM) in dermal blood vessels.
Livedoid vasculopathy can be difficult to treat. Treatment is aimed at reducing clotting and improving blood flow and includes antiplatelet drugs (low-dose aspirin, dipyridamole), anticoagulants, and vasodilating agents (nifedipine). Pentoxifylline two or three times daily may help by altering blood viscosity. A recent literature search reports success in topical dapsone applied to lesions twice daily under occlusion. Leg elevation and compression stockings help healing. Livedoid vasculopathy may have periods of activity and remission.
The case and photo were submitted by Dr. Bilu Martin.
Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/edermatologynews.com. To submit a case for possible publication, send an email to dermnews@mdedge.com
It is a vasculopathy rather than a vasculitis as the former is caused by occlusion of blood vessels and the latter results from inflammation of the vessels. Middle-aged women tend to be affected more frequently. Although the exact cause is unclear, systemic diseases, such as hypercoagulable states, may predispose vessels to develop occlusion. Associated disorders include antiphospholipid syndrome, protein C deficiency, factor V mutation, arteriosclerosis, hyperhomocysteinemia, and hepatitis C.
Typically, lesions begin as painful purpura or reticulated macules on the lower extremities that ulcerate and heal very slowly. Ankles, particularly malleoli, are more frequently affected. When they heal, they form painless white stellate scars typical of atrophie blanche. Surrounding erythema, telangiectasias, and sclerosis may be present; livedo reticularis may be seen as well.
Histologically, the epidermis may be atrophic or necrotic. Hyaline thickening of the blood vessel walls is seen. Thrombi may be present. Direct immunofluorescence of perilesional skin may be positive for complement C3 and immunoglobulin (IgM) in dermal blood vessels.
Livedoid vasculopathy can be difficult to treat. Treatment is aimed at reducing clotting and improving blood flow and includes antiplatelet drugs (low-dose aspirin, dipyridamole), anticoagulants, and vasodilating agents (nifedipine). Pentoxifylline two or three times daily may help by altering blood viscosity. A recent literature search reports success in topical dapsone applied to lesions twice daily under occlusion. Leg elevation and compression stockings help healing. Livedoid vasculopathy may have periods of activity and remission.
The case and photo were submitted by Dr. Bilu Martin.
Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/edermatologynews.com. To submit a case for possible publication, send an email to dermnews@mdedge.com
It is a vasculopathy rather than a vasculitis as the former is caused by occlusion of blood vessels and the latter results from inflammation of the vessels. Middle-aged women tend to be affected more frequently. Although the exact cause is unclear, systemic diseases, such as hypercoagulable states, may predispose vessels to develop occlusion. Associated disorders include antiphospholipid syndrome, protein C deficiency, factor V mutation, arteriosclerosis, hyperhomocysteinemia, and hepatitis C.
Typically, lesions begin as painful purpura or reticulated macules on the lower extremities that ulcerate and heal very slowly. Ankles, particularly malleoli, are more frequently affected. When they heal, they form painless white stellate scars typical of atrophie blanche. Surrounding erythema, telangiectasias, and sclerosis may be present; livedo reticularis may be seen as well.
Histologically, the epidermis may be atrophic or necrotic. Hyaline thickening of the blood vessel walls is seen. Thrombi may be present. Direct immunofluorescence of perilesional skin may be positive for complement C3 and immunoglobulin (IgM) in dermal blood vessels.
Livedoid vasculopathy can be difficult to treat. Treatment is aimed at reducing clotting and improving blood flow and includes antiplatelet drugs (low-dose aspirin, dipyridamole), anticoagulants, and vasodilating agents (nifedipine). Pentoxifylline two or three times daily may help by altering blood viscosity. A recent literature search reports success in topical dapsone applied to lesions twice daily under occlusion. Leg elevation and compression stockings help healing. Livedoid vasculopathy may have periods of activity and remission.
The case and photo were submitted by Dr. Bilu Martin.
Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/edermatologynews.com. To submit a case for possible publication, send an email to dermnews@mdedge.com
What is your diagnosis?
It most commonly affects young girls. The pathogenesis of LAHS is thought to involve a sporadic, autosomal dominant mutation that leads to a defect between the hair cuticle and the inner root sheath.1 This defect results in the hair being poorly anchored to the scalp, and therefore easily and painlessly plucked or lost during normal hair care.
The classic presentation of LAHS is that of hair thinning and hair that may be unruly and/or lackluster; the hair rarely, if ever, requires cutting.2 The key feature is the ability to easily and painlessly pluck hairs from the patient’s scalp. The affected area is limited to the scalp, and loss of eyebrows, eyelashes, and body hair should not be seen.
Diagnosis and consideration of the differential
The diagnosis of LAHS can in some cases be made on history and physical exam alone. Patients with LAHS typically will show hair thinning with or without dullness or unruliness. They lack evidence of scalp inflammation, such as erythema, scale, pruritus, and pain. Areas of hair thinning or aberration are typically not well demarcated, and there are typically not areas of complete hair loss. There is no scarring or atrophy of the scalp itself.
Diagnostic tests include the “hair pull test,” as well as trichogram testing. In the “hair pull test” a provider grasps a set of hair at the proximal shaft near the scalp. The traction applied should result in the painless and easy extraction of more than 10% of grasped hairs in a patient with LAHS. Removal of less than 10% of hair is a normal finding, as patients without LAHS typically have about 10% of their scalp hair in the telogen phase at any given time, which would result in removal during the hair pull test.3 In trichography, plucked hairs are examined under magnification, with or without the use of selective dyes. Cinnamaldehyde is a dye that stains citrulline, which is abundant in the inner root sheath, and can be a tool in identifying its presence and/or aberrations.4 A trichogram of the pulled hairs in a patient with LAHS may classically show ruffled appearance of the cuticle, misshapen anagen hair bulbs, and absence of the inner root sheath.5 Examination under magnification also allows providers to better identify telogen versus anagen hairs, which aids in the diagnosis. By carefully considering the patient history, physical exam, and results of additional hair tests, providers can make the diagnosis of LAHS and avoid unnecessary blood work and invasive procedures like scalp biopsies.
The differential diagnosis of hair loss frequently includes alopecia areata. However, in alopecia areata, patients typically have sharply demarcated areas of hair loss, which may involve the eyebrows, eyelids, and body hairs. In alopecia areata, providers may be able to identify the “exclamation point sign” in which the hair shaft thins proximally, leading to the appearance of more pigmented, thicker hairs floating above the scalp.
Telogen effluvium is a condition in which a medical illness or stress, such as systemic illness, surgery, severe emotional distress, childbirth, dietary changes, or another traumatic event, causes a disruption in the natural cycle of hair growth such that the percentage of hairs in the telogen phase increases from about 10% to up to 70%.6 Unlike in LAHS, in which shed hairs are in the anagen phase, the hair that is shed in telogen effluvium is in the telogen phase and will have a different appearance when magnified.
Anagen effluvium, loss of hairs in their growing phase, is typically associated with chemotherapy. The hairs become broken and fractured at the shaft leading to breakage at different points throughout the scalp. Affected areas can include the eyebrows, eyelashes, and body hair. In the absence of a history of administration of a chemotherapy agent (or other drug known to trigger hair loss), the diagnosis of anagen effluvium should not be made.
Patients with trichotillosis (also known as trichotillomania) present with areas of hair loss caused by intentional or subconscious hair pulling. It is considered a psychological condition that can be associated with obsessive compulsive disorder, although the presence of a secondary psychological diagnosis is not required. Providers may see irregular geometric shapes of hair loss, and on close inspection see broken hair shafts of different lengths. Patients most often pull hair from their scalps (over 70% of patients), but also can pull eyelashes, eyebrow hairs, and pubic hairs.7
Treatment
LAHS is self-limited and does not necessitate treatment. However, if patients or parents feel there is significant disease burden, perhaps with poor effects on quality of life or with psychosocial impairment, treatment with minoxidil 5% solution has been studied with some success reported in the literature.1,8,9
Ms. Natsis is a medical student at the University of California, San Diego. Dr. Eichenfield is chief of pediatric and adolescent dermatology at Rady Children’s Hospital-San Diego. He is vice chair of the department of dermatology and professor of dermatology and pediatrics at the University of California, San Diego. Ms. Natsis and Dr. Eichenfield had no relevant financial disclosures. Email them at pdnews@mdedge.com.
References
1. Arch Dermatol. 2002;138(4):501-6.
2. Int J Trichology. 2010;2(2):96-100.
3. Pediatric Dermatol. 2016:33(50):507-10.
4. Dermatol Clin. 1986;14:745-51.
5. Arch Dermatol. 2009;145(10):1123-8.
6. J Clin Diagn Res. 2015;9(9):WE01-3.
7. Am J Psychiatry. 2016;173(9):868-74.
8. Australas J Dermatol. 2018;59:e286-e287.
9. Pediatr Dermatol. 2014;31:389-90.
It most commonly affects young girls. The pathogenesis of LAHS is thought to involve a sporadic, autosomal dominant mutation that leads to a defect between the hair cuticle and the inner root sheath.1 This defect results in the hair being poorly anchored to the scalp, and therefore easily and painlessly plucked or lost during normal hair care.
The classic presentation of LAHS is that of hair thinning and hair that may be unruly and/or lackluster; the hair rarely, if ever, requires cutting.2 The key feature is the ability to easily and painlessly pluck hairs from the patient’s scalp. The affected area is limited to the scalp, and loss of eyebrows, eyelashes, and body hair should not be seen.
Diagnosis and consideration of the differential
The diagnosis of LAHS can in some cases be made on history and physical exam alone. Patients with LAHS typically will show hair thinning with or without dullness or unruliness. They lack evidence of scalp inflammation, such as erythema, scale, pruritus, and pain. Areas of hair thinning or aberration are typically not well demarcated, and there are typically not areas of complete hair loss. There is no scarring or atrophy of the scalp itself.
Diagnostic tests include the “hair pull test,” as well as trichogram testing. In the “hair pull test” a provider grasps a set of hair at the proximal shaft near the scalp. The traction applied should result in the painless and easy extraction of more than 10% of grasped hairs in a patient with LAHS. Removal of less than 10% of hair is a normal finding, as patients without LAHS typically have about 10% of their scalp hair in the telogen phase at any given time, which would result in removal during the hair pull test.3 In trichography, plucked hairs are examined under magnification, with or without the use of selective dyes. Cinnamaldehyde is a dye that stains citrulline, which is abundant in the inner root sheath, and can be a tool in identifying its presence and/or aberrations.4 A trichogram of the pulled hairs in a patient with LAHS may classically show ruffled appearance of the cuticle, misshapen anagen hair bulbs, and absence of the inner root sheath.5 Examination under magnification also allows providers to better identify telogen versus anagen hairs, which aids in the diagnosis. By carefully considering the patient history, physical exam, and results of additional hair tests, providers can make the diagnosis of LAHS and avoid unnecessary blood work and invasive procedures like scalp biopsies.
The differential diagnosis of hair loss frequently includes alopecia areata. However, in alopecia areata, patients typically have sharply demarcated areas of hair loss, which may involve the eyebrows, eyelids, and body hairs. In alopecia areata, providers may be able to identify the “exclamation point sign” in which the hair shaft thins proximally, leading to the appearance of more pigmented, thicker hairs floating above the scalp.
Telogen effluvium is a condition in which a medical illness or stress, such as systemic illness, surgery, severe emotional distress, childbirth, dietary changes, or another traumatic event, causes a disruption in the natural cycle of hair growth such that the percentage of hairs in the telogen phase increases from about 10% to up to 70%.6 Unlike in LAHS, in which shed hairs are in the anagen phase, the hair that is shed in telogen effluvium is in the telogen phase and will have a different appearance when magnified.
Anagen effluvium, loss of hairs in their growing phase, is typically associated with chemotherapy. The hairs become broken and fractured at the shaft leading to breakage at different points throughout the scalp. Affected areas can include the eyebrows, eyelashes, and body hair. In the absence of a history of administration of a chemotherapy agent (or other drug known to trigger hair loss), the diagnosis of anagen effluvium should not be made.
Patients with trichotillosis (also known as trichotillomania) present with areas of hair loss caused by intentional or subconscious hair pulling. It is considered a psychological condition that can be associated with obsessive compulsive disorder, although the presence of a secondary psychological diagnosis is not required. Providers may see irregular geometric shapes of hair loss, and on close inspection see broken hair shafts of different lengths. Patients most often pull hair from their scalps (over 70% of patients), but also can pull eyelashes, eyebrow hairs, and pubic hairs.7
Treatment
LAHS is self-limited and does not necessitate treatment. However, if patients or parents feel there is significant disease burden, perhaps with poor effects on quality of life or with psychosocial impairment, treatment with minoxidil 5% solution has been studied with some success reported in the literature.1,8,9
Ms. Natsis is a medical student at the University of California, San Diego. Dr. Eichenfield is chief of pediatric and adolescent dermatology at Rady Children’s Hospital-San Diego. He is vice chair of the department of dermatology and professor of dermatology and pediatrics at the University of California, San Diego. Ms. Natsis and Dr. Eichenfield had no relevant financial disclosures. Email them at pdnews@mdedge.com.
References
1. Arch Dermatol. 2002;138(4):501-6.
2. Int J Trichology. 2010;2(2):96-100.
3. Pediatric Dermatol. 2016:33(50):507-10.
4. Dermatol Clin. 1986;14:745-51.
5. Arch Dermatol. 2009;145(10):1123-8.
6. J Clin Diagn Res. 2015;9(9):WE01-3.
7. Am J Psychiatry. 2016;173(9):868-74.
8. Australas J Dermatol. 2018;59:e286-e287.
9. Pediatr Dermatol. 2014;31:389-90.
It most commonly affects young girls. The pathogenesis of LAHS is thought to involve a sporadic, autosomal dominant mutation that leads to a defect between the hair cuticle and the inner root sheath.1 This defect results in the hair being poorly anchored to the scalp, and therefore easily and painlessly plucked or lost during normal hair care.
The classic presentation of LAHS is that of hair thinning and hair that may be unruly and/or lackluster; the hair rarely, if ever, requires cutting.2 The key feature is the ability to easily and painlessly pluck hairs from the patient’s scalp. The affected area is limited to the scalp, and loss of eyebrows, eyelashes, and body hair should not be seen.
Diagnosis and consideration of the differential
The diagnosis of LAHS can in some cases be made on history and physical exam alone. Patients with LAHS typically will show hair thinning with or without dullness or unruliness. They lack evidence of scalp inflammation, such as erythema, scale, pruritus, and pain. Areas of hair thinning or aberration are typically not well demarcated, and there are typically not areas of complete hair loss. There is no scarring or atrophy of the scalp itself.
Diagnostic tests include the “hair pull test,” as well as trichogram testing. In the “hair pull test” a provider grasps a set of hair at the proximal shaft near the scalp. The traction applied should result in the painless and easy extraction of more than 10% of grasped hairs in a patient with LAHS. Removal of less than 10% of hair is a normal finding, as patients without LAHS typically have about 10% of their scalp hair in the telogen phase at any given time, which would result in removal during the hair pull test.3 In trichography, plucked hairs are examined under magnification, with or without the use of selective dyes. Cinnamaldehyde is a dye that stains citrulline, which is abundant in the inner root sheath, and can be a tool in identifying its presence and/or aberrations.4 A trichogram of the pulled hairs in a patient with LAHS may classically show ruffled appearance of the cuticle, misshapen anagen hair bulbs, and absence of the inner root sheath.5 Examination under magnification also allows providers to better identify telogen versus anagen hairs, which aids in the diagnosis. By carefully considering the patient history, physical exam, and results of additional hair tests, providers can make the diagnosis of LAHS and avoid unnecessary blood work and invasive procedures like scalp biopsies.
The differential diagnosis of hair loss frequently includes alopecia areata. However, in alopecia areata, patients typically have sharply demarcated areas of hair loss, which may involve the eyebrows, eyelids, and body hairs. In alopecia areata, providers may be able to identify the “exclamation point sign” in which the hair shaft thins proximally, leading to the appearance of more pigmented, thicker hairs floating above the scalp.
Telogen effluvium is a condition in which a medical illness or stress, such as systemic illness, surgery, severe emotional distress, childbirth, dietary changes, or another traumatic event, causes a disruption in the natural cycle of hair growth such that the percentage of hairs in the telogen phase increases from about 10% to up to 70%.6 Unlike in LAHS, in which shed hairs are in the anagen phase, the hair that is shed in telogen effluvium is in the telogen phase and will have a different appearance when magnified.
Anagen effluvium, loss of hairs in their growing phase, is typically associated with chemotherapy. The hairs become broken and fractured at the shaft leading to breakage at different points throughout the scalp. Affected areas can include the eyebrows, eyelashes, and body hair. In the absence of a history of administration of a chemotherapy agent (or other drug known to trigger hair loss), the diagnosis of anagen effluvium should not be made.
Patients with trichotillosis (also known as trichotillomania) present with areas of hair loss caused by intentional or subconscious hair pulling. It is considered a psychological condition that can be associated with obsessive compulsive disorder, although the presence of a secondary psychological diagnosis is not required. Providers may see irregular geometric shapes of hair loss, and on close inspection see broken hair shafts of different lengths. Patients most often pull hair from their scalps (over 70% of patients), but also can pull eyelashes, eyebrow hairs, and pubic hairs.7
Treatment
LAHS is self-limited and does not necessitate treatment. However, if patients or parents feel there is significant disease burden, perhaps with poor effects on quality of life or with psychosocial impairment, treatment with minoxidil 5% solution has been studied with some success reported in the literature.1,8,9
Ms. Natsis is a medical student at the University of California, San Diego. Dr. Eichenfield is chief of pediatric and adolescent dermatology at Rady Children’s Hospital-San Diego. He is vice chair of the department of dermatology and professor of dermatology and pediatrics at the University of California, San Diego. Ms. Natsis and Dr. Eichenfield had no relevant financial disclosures. Email them at pdnews@mdedge.com.
References
1. Arch Dermatol. 2002;138(4):501-6.
2. Int J Trichology. 2010;2(2):96-100.
3. Pediatric Dermatol. 2016:33(50):507-10.
4. Dermatol Clin. 1986;14:745-51.
5. Arch Dermatol. 2009;145(10):1123-8.
6. J Clin Diagn Res. 2015;9(9):WE01-3.
7. Am J Psychiatry. 2016;173(9):868-74.
8. Australas J Dermatol. 2018;59:e286-e287.
9. Pediatr Dermatol. 2014;31:389-90.
A 5-year-old female is brought to clinic for hair loss. The mother reports that when styling her daughter's hair, she has noticed areas of hair thinning, especially at the temples and at the occiput. The mother denies scale, pruritus, and erythema. The patient reports that her scalp does not hurt. She has never had a haircut because her hair hasn't grown long enough to cut. There is no history of specific bald spots. The patient has no personal history of psoriasis, seborrheic dermatitis, or autoimmune disease. No picking has been noted, and there is no history of compulsive behaviors or anxiety. The patient's mother has a history of Graves disease. The mother reports that the patient's older sister may have had hair thinning when she was younger as well, but no longer has thin hair.
The patient was previously seen by another provider who prescribed hydrocortisone 2.5% ointment, which the mother has been applying nightly without improvement.
The child is otherwise medically well and thriving, with no recent change in activity level and with growth parameters consistently around the 75th percentile for height and weight. On physical exam, the patient has blondish, fine hair with areas of poorly demarcated hair thinning at the left temple and at the occiput. The hair remaining at the occiput is normal in texture. There are no areas of complete hair loss. There is no scale, erythema, or abnormal pigmentation, and no cervical or occipital adenopathy. The patient has intact eyebrows and eyelashes.
What's your diagnosis?
The patient was diagnosed with lichen spinulosus (LS) based on the physical appearance of the lesions (hyperkeratotic spiny papules forming plaques), the lack of pruritus, and negative personal history of atopic dermatitis.
Lichen spinulosus is an underreported entity, first described in 1908 by Adamson as superficial circumscribed chronic dermatitis in children and adolescents. The median age of presentation is age 16 years. There are several reports of possible associations with systemic infections such as HIV, fungi, and syphilis, as well as chronic diseases such as Crohn’s disease, Hodgkin disease, seborrhea, and secondary to certain medications such as omeprazole. There are no prior reports of infliximab being associated with LS, but it has been reported to cause other lichenoid reactions such as lichen planus and lichen planopilaris.
Clinically the lesions are characterized by asymptomatic, small (1 cm), skin color, hyperkeratotic, follicular papules that coalesce into plaques. The lesions usually occur on the extensor surfaces of the arms, neck, torso, and buttocks. Mild pruritus can occur in some patients.
The lesions in keratosis pilaris can be very similar to lichen spinulosus, but usually they don’t coalesce into plaques and are commonly present on the extensor surfaces of the arms, thighs, and cheeks. Histopathology of both conditions is very similar.
Another condition to consider includes papular eczema. The lesions in papular eczema tend to be pruritic and are not as circumscribed as LS lesions. Papular eczema responds well to the use of topical corticosteroids, while LS lesions usually do not. Lichen nitidus (LN) is characterized by monomorphic, skin color, 1-mm, flat-topped papules. Lesions tend to occur in crops rather than circumscribed papules forming plaques. LN most commonly presents on the extensor surface of the arms, trunk, dorsal hands, and genitalia. Koebner phenomenon is usually seen. Although uncommon in children, a more generalized type of follicular mucinosis can present very similar to lichen spinulosus. A recent study found LS-like lesions with associated hypopigmentation and hair loss should be suspicious for folliculotropic mycosis fungoides.
Keratolytics such as lactic acid, urea, and salicylic acid can help improve LS, although they do not cure it. Other reported treatments include the use of topical adapalene, tacalcitol cream, and tretinoin gel with hydroactive adhesive.
Dr. Matiz is a pediatric dermatologist at Southern California Permanente Medical Group, San Diego. Email her at pdnews@mdedge.com.
The patient was diagnosed with lichen spinulosus (LS) based on the physical appearance of the lesions (hyperkeratotic spiny papules forming plaques), the lack of pruritus, and negative personal history of atopic dermatitis.
Lichen spinulosus is an underreported entity, first described in 1908 by Adamson as superficial circumscribed chronic dermatitis in children and adolescents. The median age of presentation is age 16 years. There are several reports of possible associations with systemic infections such as HIV, fungi, and syphilis, as well as chronic diseases such as Crohn’s disease, Hodgkin disease, seborrhea, and secondary to certain medications such as omeprazole. There are no prior reports of infliximab being associated with LS, but it has been reported to cause other lichenoid reactions such as lichen planus and lichen planopilaris.
Clinically the lesions are characterized by asymptomatic, small (1 cm), skin color, hyperkeratotic, follicular papules that coalesce into plaques. The lesions usually occur on the extensor surfaces of the arms, neck, torso, and buttocks. Mild pruritus can occur in some patients.
The lesions in keratosis pilaris can be very similar to lichen spinulosus, but usually they don’t coalesce into plaques and are commonly present on the extensor surfaces of the arms, thighs, and cheeks. Histopathology of both conditions is very similar.
Another condition to consider includes papular eczema. The lesions in papular eczema tend to be pruritic and are not as circumscribed as LS lesions. Papular eczema responds well to the use of topical corticosteroids, while LS lesions usually do not. Lichen nitidus (LN) is characterized by monomorphic, skin color, 1-mm, flat-topped papules. Lesions tend to occur in crops rather than circumscribed papules forming plaques. LN most commonly presents on the extensor surface of the arms, trunk, dorsal hands, and genitalia. Koebner phenomenon is usually seen. Although uncommon in children, a more generalized type of follicular mucinosis can present very similar to lichen spinulosus. A recent study found LS-like lesions with associated hypopigmentation and hair loss should be suspicious for folliculotropic mycosis fungoides.
Keratolytics such as lactic acid, urea, and salicylic acid can help improve LS, although they do not cure it. Other reported treatments include the use of topical adapalene, tacalcitol cream, and tretinoin gel with hydroactive adhesive.
Dr. Matiz is a pediatric dermatologist at Southern California Permanente Medical Group, San Diego. Email her at pdnews@mdedge.com.
The patient was diagnosed with lichen spinulosus (LS) based on the physical appearance of the lesions (hyperkeratotic spiny papules forming plaques), the lack of pruritus, and negative personal history of atopic dermatitis.
Lichen spinulosus is an underreported entity, first described in 1908 by Adamson as superficial circumscribed chronic dermatitis in children and adolescents. The median age of presentation is age 16 years. There are several reports of possible associations with systemic infections such as HIV, fungi, and syphilis, as well as chronic diseases such as Crohn’s disease, Hodgkin disease, seborrhea, and secondary to certain medications such as omeprazole. There are no prior reports of infliximab being associated with LS, but it has been reported to cause other lichenoid reactions such as lichen planus and lichen planopilaris.
Clinically the lesions are characterized by asymptomatic, small (1 cm), skin color, hyperkeratotic, follicular papules that coalesce into plaques. The lesions usually occur on the extensor surfaces of the arms, neck, torso, and buttocks. Mild pruritus can occur in some patients.
The lesions in keratosis pilaris can be very similar to lichen spinulosus, but usually they don’t coalesce into plaques and are commonly present on the extensor surfaces of the arms, thighs, and cheeks. Histopathology of both conditions is very similar.
Another condition to consider includes papular eczema. The lesions in papular eczema tend to be pruritic and are not as circumscribed as LS lesions. Papular eczema responds well to the use of topical corticosteroids, while LS lesions usually do not. Lichen nitidus (LN) is characterized by monomorphic, skin color, 1-mm, flat-topped papules. Lesions tend to occur in crops rather than circumscribed papules forming plaques. LN most commonly presents on the extensor surface of the arms, trunk, dorsal hands, and genitalia. Koebner phenomenon is usually seen. Although uncommon in children, a more generalized type of follicular mucinosis can present very similar to lichen spinulosus. A recent study found LS-like lesions with associated hypopigmentation and hair loss should be suspicious for folliculotropic mycosis fungoides.
Keratolytics such as lactic acid, urea, and salicylic acid can help improve LS, although they do not cure it. Other reported treatments include the use of topical adapalene, tacalcitol cream, and tretinoin gel with hydroactive adhesive.
Dr. Matiz is a pediatric dermatologist at Southern California Permanente Medical Group, San Diego. Email her at pdnews@mdedge.com.
A 7-year-old male with a history of Crohn's disease presents with 6 months of asymptomatic, bumpy lesions on the torso and extremities. He has been using over-the-counter hydrocortisone and moisturizers without it helping. His Crohn's disease has been controlled with infliximab infusions for 2 years. The mother is concerned the rash could be a side effect of the medication.
He denies any prior history of atopic dermatitis or psoriasis. The mother had eczema as a child. He has two brothers who have been diagnosed and treated for allergic rhinitis.
On physical examination, he is a thin, pleasant young boy in no distress.
His skin is somewhat dry, and there are several hyperkeratotic follicular papules forming plaques on the torso and extremities. There is no associated hair loss on the affected areas or inflammation noted.