Prevalence and pathogenesis of osteopenia and osteoporosis in epidermolysis bullosa: An evidence-based review.

Abstract

Osteopenia or osteoporosis is one of the many comorbidities in patients with Epidermolysis Bullosa (EB). Current literature on the prevalence of osteoporosis in EB is scarce. This review will analyse the current literature in the field of patients with compromised bone health in EB and any articles on the prevalence of such diseases in EB groups. A systematic search for articles related to bone health and epidermolysis bullosa (EB) (1946-2017) was performed on seven databases: MEDLINE, EMBASE and EBM, PubMed, ProQuest, Scopus and Web of Science. Search terms: epidermolysis bullosa, osteop*, bone mass, bone mineral*, fracture, dual X-ray absorptiometry, vitamin D, calcium, nutrition, exercise and physical activity. Abstracts from all search results were screened, and reference scanning of the search results was performed. Eighty-three articles met the selection criteria and were considered for review. Letters to the editor and abstract-only articles were excluded. Articles were favoured based on citation count, impact factor of their journal and study sample size. The search included all languages. The searches yielded a total of 1309 articles including 717 duplicates. The remaining 592 articles were screened by title and abstracts. Eighty-three full-text articles were analysed. Twenty-one articles directly relating to bone health in EB were included. Three descriptive studies and one case-control study were found, indicating a need for research of larger scale. Further investigations into osteoporosis in EB, especially the milder forms of EB, are valuable in providing evidence to support guideline developments for EB bone health management.