Pemphigus vulgaris presenting as esophageal ulceration. Report of an under-diagnosed manifestation

Abstract

Pemphigus vulgaris (PV) is a rare autoimmune blistering disorder of the skin and mucous membranes. The true prevalence of esophageal involvement is unknown; esophageal symptoms almost always occur in the context of oral mucosa involvement. We report the case of a 66-year-old man with cutaneous blisters and esophageal symptoms that did not respond to acid suppression therapy. Esophagogastroduodenoscopy showed esophageal ulcers and mucosal desquamation. Biopsies were consistent with the diagnosis of PV. The patient was started on immunosuppressive therapy, achieving remission. This represents a rare case of esophageal involvement of PV without mucosal involvement and draws attention to a rare cause of dysphagia, which can be fatal if left untreated.