Onychodystrophy and Scarring Alopecia in Epidermolysis Bullosa Acquisita: A Case Based Review of the Literature

Introduction: Epidermolysis bullosa acquisita is a rare, autoimmune subepidermal bullous disease. Besides blisters appearance which heals with scars and milia cysts formation, nails undergo different changes: onychomadesis, onycholysis, onychorrhexis and matrix destruction. Case report: We present the case of a 59 years old patient, which showed multiple blisters, erosions, crusts located on the elbows, knees, hands, and feet; some lesions healed and formed hypo or hyperpigmented. scars in the evolution. On the scalp were observed areas of scar alopecia. In the oral cavity there were extensive erosions, painful dysphagia to both solid and liquid food, which in time contributed to significant weight loss. To the fingers and toes, the patient underwent nail loss, these being to be replaced with scar tissue. The only remaining nails are at the fourth and fifth fingers of both hands, but also those showing lesions of onycholysis and onychorrhexis leading to a thin epidermal nail. Conclusions: Our case report is the third case described in the literature. Onychomadesis manifestations and nail absence, although rare, complete the cutaneous manifestations of Epidermolysis bullosa acquisita.