Background: Osteopenia and osteoporosis are one of the many comorbidities in patients with Epidermolysis Bullosa (EB). Currently there is limited data on the prevalence of osteopenia and osteoporosis in patients with EB.
Objective: To discuss and analyse the current literature in the field of patients with compromised bone health in EB, and more specifically any articles on the prevalence of such diseases in EB groups.
Methods: A systematic search for articles related to bone health and EB published until 2017 was performed on six databases: Medline, Embase, PubMed, ProQuest,Scopus and Web of Science. The following search terms were used: epidermolysis bullosa, osteop*, bone mass, bone mineral*, fracture, dual x-ray absorptiometry, vitamin d, calcium, nutrition, exercise and physical activity. The Boolean command ‘AND’ was used to create searches relevant to the research question. Of the total 1309 search results, 592 articles remained after duplicates were removed and 83 articles were deemed relevant after screening of the titles and abstracts. In addition, reference scanning of the search results was performed. These articles were systematically analysed and critically evaluated. Exclusion criteria of irrelevant papers consisted of any letters to the editor and abstract-only articles. Articles were favoured based on citation count, journal impact factor and relevance to the research question. The search did not restrict to English-only articles.
Results: Included studies were articles that pertained to bone health and all types of EB. Three descriptive studies and one case control study were found, indicating a need for research of larger scale.
Conclusion: Further investigations into osteopenia and osteoporosis in EB, especially the milder forms of EB, such as epidermolysis bullosa simplex and dominant dystrophic EB, is of considerable value in providing evidence to support the development of guidelines for monitoring and managing bone health in patients with EB.