Pemphigus vulgaris presenting as esophageal ulceration. Report of an underdiagnosed manifestation

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

Esophageal Pemphigus Vulgaris: A Rare Etiology of Upper Gastrointestinal Hemorrhage

Pemphigus vulgaris (PV) is an autoimmune blistering disorder of the skin and mucosal surfaces characterized by acantholysis (loss of adhesion between epidermal cells). Esophageal involvement of PV is an underdiagnosed entity as routine diagnostic endoscopy is not recommended in asymptomatic patients. Dysphagia and odynophagia are common presenting symptoms; however, upper gastrointestinal bleeding (UGIB) associated with esophageal involvement of PV without a history of mucosal blistering is extremely uncommon. We present a case of esophageal involvement of PV associated with active UGIB that was diagnosed on endoscopic evaluation. This case illustrated the importance of early endoscopy to identify the esophageal involvement of PV especially in patients with preexisting disease who present with gastrointestinal symptoms such as dysphagia, odynophagia, and hematemesis. Early recognition of esophageal involvement of PV and initiation of corticosteroid and/or immunosuppressant therapy may improve the outcome of the disease.

Endoluminal Vacuum Therapy for Iatrogenic Perforation of the Proximal Esophagus—Case Report of a Patient with Esophageal Involvement in Pemphigus Vulgaris

Pemphigus vulgaris (PV) is a rare autoimmune disease affecting the skin and mucous membranes. Recent studies have shown a relatively high incidence of esophageal involvement in PV. To distinguish between the esophageal involvement of PV and other pathologies, early endoscopic evaluation is essential to obtain tissue for histological analysis. Here, we present the first case of endoluminal vacuum therapy (EVT) for a patient with esophageal involvement in PV and with associated iatrogenic perforation of the proximal esophagus. In comparison to classical treatment options for perforations of the proximal esophagus consisting of either stent insertion or surgical intervention, EVT seems to be a promising alternative with a good clinical outcome.