Epidermolysis bullosa acquisita: an uncommon cause of esophageal stricture

Epidermolysis bullosa acquisita (EBA) encompasses a wide spectrum of rare diseases with a common genetic origin transmitted in an autosomal recessive fashion. Mild forms of non-inflammatory EBA are characterized by skin lesions and have gained great relevance in the literature. However, resistant inflammatory EBA with widespread mucosal involvement remains a rare entity given its low prevalence. It commonly represents a great burden for the patient’s quality of life with most cases being resistant to different therapeutic modalities. We present a case of resistant inflammatory EBA with esophageal strictures that improved after therapy with intravenous immunoglobulin and rituximab.