Introduction

Acquired epidermolysis bullosa (AEB) is a rare autoimmune subepidermal blistering disorder mediated by autoantibodies against type VII collagen. Mucosal lesions are frequent, but esophageal involvement remains exceptional. When present, it may cause progressive dysphagia linked to cicatricial strictures, with major nutritional impact and impaired quality of life.

Case Report

A 50-year-old man presented with bullous and post-bullous lesions on friction areas, associated with atrophic scarring and milia. Oral and nasal mucosal erosions were noted, with an 8-kg weight loss. Histopathology showed a subepidermal blister; direct immunofluorescence revealed linear IgG/C3 at the dermo-epidermal junction. ELISA demonstrated markedly elevated anti–type VII collagen antibodies and marginal anti-envoplakin positivity.Corticosteroids (0.5 mg/kg/day) were ineffective. Dapsone (100 mg/day) produced partial cutaneous improvement only. Endoscopy revealed a 7-cm circumferential mid-esophageal ulceration. Cyclosporine (3 mg/kg/day) induced transient improvement followed by progressive dysphagia.Repeat endoscopy showed an ulcerated cervical stricture with extensive mucosal detachment. Balloon dilation combined with high-dose corticosteroids and IVIG restored swallowing, improved weight, and prevented recurrence despite persistent mucosal fragility. Rituximab was subsequently initiated.

Discussion

Esophageal involvement in AEB, although rare, represents a severe mucosal manifestation. Blistering, ulceration, and fibrotic strictures may occur, and endoscopy—while essential—is technically hazardous due to high mucosal fragility. Conventional therapies frequently fail to control severe mucosal disease. Endoscopic dilation is often required but carries procedural risks.Recent literature supports IVIG and rituximab as effective options in severe, refractory AEB, including stenosing esophageal forms, with favorable tolerance profiles.

Conclusion

Esophageal AEB necessitates individualized, multidisciplinary management. Biologic therapies offer promising perspectives for refractory and stenosing mucosal disease.