Faculty of Medicine, University of New South Wales, Sydney, NSW, Australia.
Department of Dermatology, St George Hospital, Sydney, NSW, Australia.
Exp Dermatol. 2021 Nov;30(11):1675-1685. doi: 10.1111/exd.14252. Epub 2021 Jan 10.
Epidermolysis bullosa (EB) patients have multiple risk factors for osteoporosis. There is limited literature describing the prevalence of bone health in EB, particularly in adults and less severe EB types.
To investigate the prevalence of osteopenia or osteoporosis in EB patients from the Australasian Epidermolysis Bullosa Registry (AEBR).
Of 417 AEBR patients, 72 underwent a dual energy X-ray absorptiometry scan. Bone mineral density (BMD) T and Z-scores, EB Disease Activity and Scarring Index (EBDASI), and Quality of Life in EB (QOLEB) scores were obtained.
T-scores of RDEB patients were significantly lower than the diagnostic cut-off value for osteopenia. EBDASI and QOLEB scores were inversely correlated with Z-scores. The prevalence of osteoporosis in adults was 75% in severe EB types (RDEB and JEB). In adults with less severe types (EBS and DDEB), the prevalence of osteopenia was 50% and 33%, respectively.
This is the largest study of osteoporosis in EB to date and the first to include adult patients with EBS. The high prevalence of osteoporosis and osteopenia identified in these patients warrants larger, collaborative international studies. Nevertheless, EB patients with high disease severity and QOL scores, irrespective of type, should receive early osteoporosis screening and prophylaxis.
大疱性表皮松解症（EB）患者存在多种骨质疏松症风险因素。关于 EB 患者的骨骼健康状况，尤其是成人和病情较轻的 EB 类型，相关文献有限。
调查澳大利亚大疱性表皮松解症登记处（AEBR） EB 患者中骨量减少或骨质疏松症的患病率。
在 417 名 AEBR 患者中，有 72 名患者接受了双能 X 线吸收法扫描。获得了骨矿物质密度（BMD）T 和 Z 评分、EB 疾病活动和瘢痕指数（EBDASI）以及 EB 生活质量（QOLEB）评分。
RDEB 患者的 T 评分明显低于骨质疏松症的诊断临界值。EBDASI 和 QOLEB 评分与 Z 评分呈负相关。严重 EB 类型（RDEB 和 JEB）中成人骨质疏松症的患病率为 75%。在病情较轻的成人类型（EBS 和 DDEB）中，骨量减少的患病率分别为 50%和 33%。
这是迄今为止 EB 骨质疏松症的最大研究，也是首次纳入 EBS 成人患者的研究。这些患者中发现的骨质疏松症和骨量减少的高患病率需要更大规模的、协作性的国际研究。尽管如此，无论类型如何，具有高疾病严重程度和 QOL 评分的 EB 患者都应接受早期骨质疏松症筛查和预防。
