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TOPLINE:

Authors highlight the effects of epidermolysis bullosa (EB) on bone health and call for improved research and development of clinical practice guidelines.

METHODOLOGY:

• Osteoporosis results in considerable burden in pediatric patients with EB, but data on the prevalence and events preceding bone loss in this population are limited.
• To characterize the prevalence of osteoporosis and bone health in pediatric patients with EB and to summarize clinical practice recommendations on the topic, researchers conducted a scoping review of 21 studies in the literature on impaired nutrition or any factors that affect bone health in this population.
• Studies published in languages other than English were excluded from the analysis, as were abstracts and review articles.

TAKEAWAY:

• The researchers summarized recommendations from 13 of the 21 studies, which were published during 1971-2022.
• Among the recommendations were obtaining nutritional and weight assessments before the age of 2 years, blood tests every 6-12 months starting at birth, and Tanner stage assessments every 6 months.
• Other recommendations included using dual-energy x-ray absorptiometry scans starting at the age of 6 years, with subsequent scans every 1-2 years, except in mild cases, and supplementation with vitamin D (80-320 IU daily for children aged 0-7 years and 720 IU for patients older than 8 years).

IN PRACTICE:

"Given the complexity affecting bone health and the impact of poor bone health on quality of life for children, there is a significant unmet need for mechanistic research of the molecular impact of EB on bone metabolism as well as the development of guidelines for screening, monitoring, and treating osteoporosis in EB," the researchers wrote. 

STUDY DETAILS:

Amy S. Paller, MD, chair of the department of dermatology at Northwestern University, Chicago, led the research. The study was published online on January 11, 2024, in Pediatric Dermatology.

LIMITATIONS:

The small sample sizes of the studies, lack of homogeneity in the study populations, and the use of different measures of bone health outcome. 

DISCLOSURES:

The study received funding support from the Pediatric Dermatology Research Alliance and the German Research Foundation. The authors reported having no financial disclosures.