Target plantar keratoderma when managing ‘mild’ EBS

LONDON – Hardened feet are a major determinant of the clinical course of epidermolysis bullosa simplex (EBS), according to research presented by a German team of investigators at the EB World Congress.

In a study of 157 individuals with EBS, 75.8% had plantar keratoderma, a condition associated with a vicious circle of pain, reduced mobility, subsequent weight gain, and further foot problems.

“EBS has severe impacts on various aspects of everyday life,” Antonia Reimer, MD, and associates at the University of Freiburg, Germany, reported in a poster presentation. “Plantar involvement and [plantar keratoderma] are serious complications of all EBS subtypes, correlating with excessive weight gain, pain, local infections, and limited mobility.”

The researchers suggested that “targeting [plantar keratoderma] should be a priority in EBS therapy and research.”

In their retrospective cohort study, clinical and molecular data were retrieved from patient records, and major determinants of the clinical course of EBS investigated. As such, the researchers looked at how weight changes affected EBS, the effect of hardening skin on the feet, pain, mobility, and working life.

“EB simplex is generally regarded as the ‘mildest’ EB type,” Dr. Reimer and colleagues wrote, “however, individuals with EBS report a high disease burden and frequent pain.” The team found that just under 30% of patients (n = 46) experienced frequent pain, particularly those with localized and severe EBS. Of the patients experiencing pain, the majority (75.2%) had plantar keratoderma. Furthermore, those with blisters underneath the hardened skin reported having the most painful lesions.

Palmoplantar hyperhidrosis was present in slightly more than 40% of cases, and was especially common in individuals with localized EBS, Dr. Reimer and colleagues found. They also found that bacterial and fungal infections occurred in 14% and 7% of patients, respectively, and this correlated significantly with diffuse plantar keratoderma.

A third of patients experience mobility problems, and 8.2% required a wheelchair; 16.4% “were in occupational disability,” the team reported.

“Hyperkeratosis is important because it isn’t just about treating the hyperkeratosis, it’s also looking at the mechanical balance of the foot,” Tariq Khan, PhD, said during an unrelated oral presentation. Dr. Khan, a consultant podiatrist specializing in EB at Great Ormond Street Hospital NHS Foundation Trust in London, discussed how to best manage the feet of people with EB.

“Podiatry technology and how we treat can often be detrimental to an EB patient,” Dr. Khan cautioned at the meeting, which was organized by the Dystrophic Epidermolysis Bullosa Association (DEBRA).

For example, “certain devices, certain types of material, will add more friction and pressure and cause more blistering,” he added, making treatment challenging.

 

Having worked with the EB community for the past 22 years, he noted that he had seen how podiatry practices had been refined to deal with this patient population. Dr. Khan is one of several experts behind EB podiatry guidelines issued by DEBRA International last year (Br J Dermatol. 2019 Aug 9. doi: 10.1111/bjd.18381) and has run the charity’s first practical EB podiatry skills course to educate more podiatrists on the intricacies of managing EB feet.

During his talk, Dr. Kahn mentioned several innovations that came about by working with external companies, such as the production of special cotton socks containing silver fibers to help reduce the symptom of hot feet, and development of a cooling insole that helped draw moisture and odor away from the foot while providing comfort to the wearer.

One of the main problems for those with EB is finding comfortable footwear that doesn’t aggravate their symptoms, Dr. Khan emphasized.

According to the EB podiatry guidelines, footwear needs to be supportive, and “its primary focus should be aimed at minimizing blistering by reducing friction.” If blisters are already present, the guidelines note that dressings and topical antiseptics or antibiotics might be used until the blisters heal. “Therefore, suitable shoes or footwear are essential to accommodate dressings and not lead to further trauma to the damaged area. Footwear that is adjustable may be beneficial in these circumstances.”

What constitutes appropriate footwear is open to debate and was the topic of a separate poster presentation at meeting. Mark O’Sullivan, EB team podiatrist at Solihull and Birmingham Women’s and Children’s NHS Foundation Trust, and associates looked at whether wearing rocker bottom footwear could ease the formation of blisters in patients with EBS.

The team studied nine patients who reported regular plantar blistering. An in-shoe measurement system was devised to measure patients’ plantar pressure while they were wearing their existing footwear and then again when they were wearing new footwear with a rocker bottom. Participants completed questionnaires about the development of blisters on their feet, their activity levels, and pain.

The rocker bottom footwear reduced the peak plantar pressure by 30.5% and the total plantar pressure by 31.8%, compared with regular footwear. A shift in the average pressure under the foot was seen, moving from the heels of the feet to the midfoot area, while remaining similar in the front foot area.

“Patient feedback has been mixed,” Mr. O’Sullivan said when presenting the poster. “Patients state that blisters have often reduced in the heels and forefoot, but new blisters have developed in the midfoot.” As a result, some study participants chose to alternate wearing the rocker bottom footwear with their normal shoes, to even out the places where blisters might form.

 

Although the jury is still out on the benefit of rocker bottom footwear, one thing that might help those with EBS who develop regular foot blisters may be to keep their weight in check. In a separate poster presentation given by Lynn Hubbard, a specialist EB dietitian in the department of nutrition and dietetics at St. Thomas’ Hospital in London, it was shown that almost a third of patients with EB simplex were obese, compared with 26% of adults in the general U.K. population.

“People with EBS are known to have hyperkeratosis and foot-blistering,” Ms. Hubbard observed in the poster. This can lead to reduce mobility and pain, which “may in turn have an impact on body weight, and an increased BMI [body mass index] may further affect mobility.”

Data were collected on 90 patients who attended a U.K. EBS clinic over an 11-month period. While 45.5% of patients had a normal weight, the majority was overweight (21.1%), obese (21.1%), or morbidly obese (10%).

Fifteen patients completed questionnaires about their mobility, and almost all felt that their weight had an adverse effect on their feet, as did EBS. Several also noted problems with their EBS, in the skin folds around the bra, waist, and sock lines.

“We now plan to begin a pilot study to establish a supportive weight management program for people with EBS and evaluate both weight loss and impact on mobility,” Ms. Hubbard reported.

No conflicts of interest were declared by any of the speakers.

SOURCES: EB 2020. Reimer A et al. Poster 26; Khan T. oral presentation; O’Sullivan M et al. Poster 93; Hubbard L. Poster 19.

LONDON – Hardened feet are a major determinant of the clinical course of epidermolysis bullosa simplex (EBS), according to research presented by a German team of investigators at the EB World Congress.

In a study of 157 individuals with EBS, 75.8% had plantar keratoderma, a condition associated with a vicious circle of pain, reduced mobility, subsequent weight gain, and further foot problems.

“EBS has severe impacts on various aspects of everyday life,” Antonia Reimer, MD, and associates at the University of Freiburg, Germany, reported in a poster presentation. “Plantar involvement and [plantar keratoderma] are serious complications of all EBS subtypes, correlating with excessive weight gain, pain, local infections, and limited mobility.”

The researchers suggested that “targeting [plantar keratoderma] should be a priority in EBS therapy and research.”

In their retrospective cohort study, clinical and molecular data were retrieved from patient records, and major determinants of the clinical course of EBS investigated. As such, the researchers looked at how weight changes affected EBS, the effect of hardening skin on the feet, pain, mobility, and working life.

“EB simplex is generally regarded as the ‘mildest’ EB type,” Dr. Reimer and colleagues wrote, “however, individuals with EBS report a high disease burden and frequent pain.” The team found that just under 30% of patients (n = 46) experienced frequent pain, particularly those with localized and severe EBS. Of the patients experiencing pain, the majority (75.2%) had plantar keratoderma. Furthermore, those with blisters underneath the hardened skin reported having the most painful lesions.

Palmoplantar hyperhidrosis was present in slightly more than 40% of cases, and was especially common in individuals with localized EBS, Dr. Reimer and colleagues found. They also found that bacterial and fungal infections occurred in 14% and 7% of patients, respectively, and this correlated significantly with diffuse plantar keratoderma.

A third of patients experience mobility problems, and 8.2% required a wheelchair; 16.4% “were in occupational disability,” the team reported.

“Hyperkeratosis is important because it isn’t just about treating the hyperkeratosis, it’s also looking at the mechanical balance of the foot,” Tariq Khan, PhD, said during an unrelated oral presentation. Dr. Khan, a consultant podiatrist specializing in EB at Great Ormond Street Hospital NHS Foundation Trust in London, discussed how to best manage the feet of people with EB.

“Podiatry technology and how we treat can often be detrimental to an EB patient,” Dr. Khan cautioned at the meeting, which was organized by the Dystrophic Epidermolysis Bullosa Association (DEBRA).

For example, “certain devices, certain types of material, will add more friction and pressure and cause more blistering,” he added, making treatment challenging.

 

Having worked with the EB community for the past 22 years, he noted that he had seen how podiatry practices had been refined to deal with this patient population. Dr. Khan is one of several experts behind EB podiatry guidelines issued by DEBRA International last year (Br J Dermatol. 2019 Aug 9. doi: 10.1111/bjd.18381) and has run the charity’s first practical EB podiatry skills course to educate more podiatrists on the intricacies of managing EB feet.

During his talk, Dr. Kahn mentioned several innovations that came about by working with external companies, such as the production of special cotton socks containing silver fibers to help reduce the symptom of hot feet, and development of a cooling insole that helped draw moisture and odor away from the foot while providing comfort to the wearer.

One of the main problems for those with EB is finding comfortable footwear that doesn’t aggravate their symptoms, Dr. Khan emphasized.

According to the EB podiatry guidelines, footwear needs to be supportive, and “its primary focus should be aimed at minimizing blistering by reducing friction.” If blisters are already present, the guidelines note that dressings and topical antiseptics or antibiotics might be used until the blisters heal. “Therefore, suitable shoes or footwear are essential to accommodate dressings and not lead to further trauma to the damaged area. Footwear that is adjustable may be beneficial in these circumstances.”

What constitutes appropriate footwear is open to debate and was the topic of a separate poster presentation at meeting. Mark O’Sullivan, EB team podiatrist at Solihull and Birmingham Women’s and Children’s NHS Foundation Trust, and associates looked at whether wearing rocker bottom footwear could ease the formation of blisters in patients with EBS.

The team studied nine patients who reported regular plantar blistering. An in-shoe measurement system was devised to measure patients’ plantar pressure while they were wearing their existing footwear and then again when they were wearing new footwear with a rocker bottom. Participants completed questionnaires about the development of blisters on their feet, their activity levels, and pain.

The rocker bottom footwear reduced the peak plantar pressure by 30.5% and the total plantar pressure by 31.8%, compared with regular footwear. A shift in the average pressure under the foot was seen, moving from the heels of the feet to the midfoot area, while remaining similar in the front foot area.

“Patient feedback has been mixed,” Mr. O’Sullivan said when presenting the poster. “Patients state that blisters have often reduced in the heels and forefoot, but new blisters have developed in the midfoot.” As a result, some study participants chose to alternate wearing the rocker bottom footwear with their normal shoes, to even out the places where blisters might form.

 

Although the jury is still out on the benefit of rocker bottom footwear, one thing that might help those with EBS who develop regular foot blisters may be to keep their weight in check. In a separate poster presentation given by Lynn Hubbard, a specialist EB dietitian in the department of nutrition and dietetics at St. Thomas’ Hospital in London, it was shown that almost a third of patients with EB simplex were obese, compared with 26% of adults in the general U.K. population.

“People with EBS are known to have hyperkeratosis and foot-blistering,” Ms. Hubbard observed in the poster. This can lead to reduce mobility and pain, which “may in turn have an impact on body weight, and an increased BMI [body mass index] may further affect mobility.”

Data were collected on 90 patients who attended a U.K. EBS clinic over an 11-month period. While 45.5% of patients had a normal weight, the majority was overweight (21.1%), obese (21.1%), or morbidly obese (10%).

Fifteen patients completed questionnaires about their mobility, and almost all felt that their weight had an adverse effect on their feet, as did EBS. Several also noted problems with their EBS, in the skin folds around the bra, waist, and sock lines.

“We now plan to begin a pilot study to establish a supportive weight management program for people with EBS and evaluate both weight loss and impact on mobility,” Ms. Hubbard reported.

No conflicts of interest were declared by any of the speakers.

SOURCES: EB 2020. Reimer A et al. Poster 26; Khan T. oral presentation; O’Sullivan M et al. Poster 93; Hubbard L. Poster 19.

LONDON – Hardened feet are a major determinant of the clinical course of epidermolysis bullosa simplex (EBS), according to research presented by a German team of investigators at the EB World Congress.

In a study of 157 individuals with EBS, 75.8% had plantar keratoderma, a condition associated with a vicious circle of pain, reduced mobility, subsequent weight gain, and further foot problems.

“EBS has severe impacts on various aspects of everyday life,” Antonia Reimer, MD, and associates at the University of Freiburg, Germany, reported in a poster presentation. “Plantar involvement and [plantar keratoderma] are serious complications of all EBS subtypes, correlating with excessive weight gain, pain, local infections, and limited mobility.”

The researchers suggested that “targeting [plantar keratoderma] should be a priority in EBS therapy and research.”

In their retrospective cohort study, clinical and molecular data were retrieved from patient records, and major determinants of the clinical course of EBS investigated. As such, the researchers looked at how weight changes affected EBS, the effect of hardening skin on the feet, pain, mobility, and working life.

“EB simplex is generally regarded as the ‘mildest’ EB type,” Dr. Reimer and colleagues wrote, “however, individuals with EBS report a high disease burden and frequent pain.” The team found that just under 30% of patients (n = 46) experienced frequent pain, particularly those with localized and severe EBS. Of the patients experiencing pain, the majority (75.2%) had plantar keratoderma. Furthermore, those with blisters underneath the hardened skin reported having the most painful lesions.

Palmoplantar hyperhidrosis was present in slightly more than 40% of cases, and was especially common in individuals with localized EBS, Dr. Reimer and colleagues found. They also found that bacterial and fungal infections occurred in 14% and 7% of patients, respectively, and this correlated significantly with diffuse plantar keratoderma.

A third of patients experience mobility problems, and 8.2% required a wheelchair; 16.4% “were in occupational disability,” the team reported.

“Hyperkeratosis is important because it isn’t just about treating the hyperkeratosis, it’s also looking at the mechanical balance of the foot,” Tariq Khan, PhD, said during an unrelated oral presentation. Dr. Khan, a consultant podiatrist specializing in EB at Great Ormond Street Hospital NHS Foundation Trust in London, discussed how to best manage the feet of people with EB.

“Podiatry technology and how we treat can often be detrimental to an EB patient,” Dr. Khan cautioned at the meeting, which was organized by the Dystrophic Epidermolysis Bullosa Association (DEBRA).

For example, “certain devices, certain types of material, will add more friction and pressure and cause more blistering,” he added, making treatment challenging.

 

Having worked with the EB community for the past 22 years, he noted that he had seen how podiatry practices had been refined to deal with this patient population. Dr. Khan is one of several experts behind EB podiatry guidelines issued by DEBRA International last year (Br J Dermatol. 2019 Aug 9. doi: 10.1111/bjd.18381) and has run the charity’s first practical EB podiatry skills course to educate more podiatrists on the intricacies of managing EB feet.

During his talk, Dr. Kahn mentioned several innovations that came about by working with external companies, such as the production of special cotton socks containing silver fibers to help reduce the symptom of hot feet, and development of a cooling insole that helped draw moisture and odor away from the foot while providing comfort to the wearer.

One of the main problems for those with EB is finding comfortable footwear that doesn’t aggravate their symptoms, Dr. Khan emphasized.

According to the EB podiatry guidelines, footwear needs to be supportive, and “its primary focus should be aimed at minimizing blistering by reducing friction.” If blisters are already present, the guidelines note that dressings and topical antiseptics or antibiotics might be used until the blisters heal. “Therefore, suitable shoes or footwear are essential to accommodate dressings and not lead to further trauma to the damaged area. Footwear that is adjustable may be beneficial in these circumstances.”

What constitutes appropriate footwear is open to debate and was the topic of a separate poster presentation at meeting. Mark O’Sullivan, EB team podiatrist at Solihull and Birmingham Women’s and Children’s NHS Foundation Trust, and associates looked at whether wearing rocker bottom footwear could ease the formation of blisters in patients with EBS.

The team studied nine patients who reported regular plantar blistering. An in-shoe measurement system was devised to measure patients’ plantar pressure while they were wearing their existing footwear and then again when they were wearing new footwear with a rocker bottom. Participants completed questionnaires about the development of blisters on their feet, their activity levels, and pain.

The rocker bottom footwear reduced the peak plantar pressure by 30.5% and the total plantar pressure by 31.8%, compared with regular footwear. A shift in the average pressure under the foot was seen, moving from the heels of the feet to the midfoot area, while remaining similar in the front foot area.

“Patient feedback has been mixed,” Mr. O’Sullivan said when presenting the poster. “Patients state that blisters have often reduced in the heels and forefoot, but new blisters have developed in the midfoot.” As a result, some study participants chose to alternate wearing the rocker bottom footwear with their normal shoes, to even out the places where blisters might form.

 

Although the jury is still out on the benefit of rocker bottom footwear, one thing that might help those with EBS who develop regular foot blisters may be to keep their weight in check. In a separate poster presentation given by Lynn Hubbard, a specialist EB dietitian in the department of nutrition and dietetics at St. Thomas’ Hospital in London, it was shown that almost a third of patients with EB simplex were obese, compared with 26% of adults in the general U.K. population.

“People with EBS are known to have hyperkeratosis and foot-blistering,” Ms. Hubbard observed in the poster. This can lead to reduce mobility and pain, which “may in turn have an impact on body weight, and an increased BMI [body mass index] may further affect mobility.”

Data were collected on 90 patients who attended a U.K. EBS clinic over an 11-month period. While 45.5% of patients had a normal weight, the majority was overweight (21.1%), obese (21.1%), or morbidly obese (10%).

Fifteen patients completed questionnaires about their mobility, and almost all felt that their weight had an adverse effect on their feet, as did EBS. Several also noted problems with their EBS, in the skin folds around the bra, waist, and sock lines.

“We now plan to begin a pilot study to establish a supportive weight management program for people with EBS and evaluate both weight loss and impact on mobility,” Ms. Hubbard reported.

No conflicts of interest were declared by any of the speakers.

SOURCES: EB 2020. Reimer A et al. Poster 26; Khan T. oral presentation; O’Sullivan M et al. Poster 93; Hubbard L. Poster 19.