Scarring Alopecia in Dystrophic Epidermolysis Bullosa: A Case Report and Scoping Review

Summary: Alopecia is a known complication of the epidermolysis bullosa (EB) group of blistering diseases. While diffuse, scarring alopecia is recognized in severe generalized EB, there is a dearth of literature characterizing alopecia in localized EB, with few reports of scarring and nonscarring alopecia in dystrophic EB. We present the case of a 58-year-old female with eight-year history of pretibial dystrophic EB who presented to dermatology clinic with improving skin fragility on five months of topical beremagene geperpavec therapy now noting gradual hair thinning on the vertex scalp over one year. Clinical examination noted decreased hair density on the vertex and frontal scalp with scattered sclerotic papules without significant perifollicular scale or erythema. Two four-millimeter punch biopsies were obtained for histologic vertical and horizontal sections, revealing superficial and mid dermal fibrosis encircling several hair follicles at the infundibulum and isthmus, with numerous fibrous stelae, a superficial perifollicular lymphocytic infiltrate with scattered follicular dyskeratotic keratinocytes, and preservation of sebaceous glands. Together, these findings were consistent with lymphocytic, scarring alopecia with shared features of dystrophic EB and early lichen planopilaris. We highlight the importance of recognizing alopecia as a known complication of dystrophic EB with discernment for concomitant inflammatory etiologies that may co-occur.