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Abstract

Background: Hereditary epidermolysis bullosa (EB) is a genetic disorder affecting skin adhesion proteins, which can impact hair follicle integrity, leading to alopecia. Objectives: We aimed to describe the epidemiological and clinical characteristics of alopecia in EB and its correlation with EB subtypes. Methods: Patients with EB were selected retrospectively and data on alopecia were collected. Results: Among 52 EB patients (M/F ratio = 1.08) with a mean age of 18.8 years (5 months to 65 years), subtypes included recessive dystrophic EB, severe (RDEB-sev) (57.7%), recessive dystrophic EB, intermediate (RDEB-int) (25%), dominant dystrophic EB (DDEB) (7.7%), autosomal dominant EB simplex, severe (AD-EBS-sev) (5.7%), and dystrophic EB pruriginosa (DEB-prur) (3.8%). Alopecia was observed in 63.5% of cases, predominantly in females (F/M ratio = 1.75). It was present in 80% of RDEB-sev cases (40% scarring alopecia (SA), 60% non-scarring alopecia (NSA), and 20% mixed), 46.6% of RDEB-int cases (42.8% SA, 57.1% NSA), and 66.6% of AD-EBS-sev cases (all NSA, with one psoriasiform plaque). The average onset was at 4.2 years, and alopecia primarily affected the frontal area (75.8%). Trichoscopy, performed in 3 patients, showed an alternation of white and pigmented bands and perifollicular hyperkeratosis in 2 patients with RDEB-int with SA. Conclusions: EB frequently leads to alopecia, particularly SA in dystrophic EB. However, in this series, dystrophic EB more commonly presented with NSA, potentially due to anemia or intrinsic hair abnormalities. DDEB was associated with NSA, though no scalp involvement was noted in this study. Trichoscopy revealed varied findings across cases, suggesting it may help distinguish alopecia types in EB.

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