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

Abstract

Background 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. Objective 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. Methods 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. Results 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. Conclusion 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.

Cannabinoid use and effects in patients with epidermolysis bullosa: an international cross-sectional survey study

“…These respondents reported both improved energy levels and ability to move around . It is likely that these improvement are related to the reduction in symptomatic burdens, yet remains an important finding as increased mobility in EB has myriad conceivable benefits [ 63 – 65 ].…”

Prevalence and treatment of vitamin K deficiency in paediatric patients with recessive dystrophic epidermolysis bullosa‐severe subtype

“…These findings confirmed the suspicion that patients with RDEB‐S are at high risk for this deficiency due to their compromised oral intake, chronic malnutrition and likely secondary gut dysbiosis due to frequent antibiotic prescription and slow transit time 15,16 . In addition, patients with RDEB‐S can commonly display poor bone mineral density; suffering from fractures and osteopenia/osteoporosis 3–5,17 . It is well documented that patients with RDEB‐S are at high risk of both vitamin D deficiency and from the negative impact of chronic inflammation on bone mineral density 17 ; however, it is as yet unknown whether chronic vitamin K deficiency may also play a contributing role in this chronic bone disease.…”

Effect of estradiol on high glucose‑induced osteoblast injury

“…In patients with type 2 diabetes, approximately one-half to two-thirds of the cases are associated with a decrease in bone density, and one-third of the patients are diagnosed with osteoporosis (OP) (2). OP is a systemic bone disease characterized by bone density loss and bone microstructural changes, which results in increased bone fragility, decreased bone strength and increased risk of fracture (3,4). Estradiol (E2) is a key factor in the development of OP, with evidence of significant bone loss in postmenopausal women (5,6).…”