Mucous Membrane Pemphigoid: A Rare Cause of Dysphagia

Abstract

A 74 year old female with a history of presumed bullous pemphigoid (BP), presented with a few cutaneous blisters, multiple oral blisters, odynophagia, dysphagia to solids and liquids and 2 days of spitting blood. An upper endoscopy (EGD) showed severe inflammation, denuded and blistering esophageal mucosa that easily bled on contact that involved the entire esophagus. Biopsies were not performed but there was high suspicion that this was esophageal involvement with BP. The patient was started on pantoprazole twice daily, liquid carafate, viscous lidocaine and later was started on prednisone and mycophenolate. Two months later the patient reported resolution of her odynophagia but continued to have dysphagia and poor oral intake. Repeat EGD showed marked improvement of the esophagus but she continued to have friable esophageal tissue that bled on contact and scar formation. Biopsies revealed benign squamous mucosa, no evidence of infectious organisms, dysplasia or malignancy. Due to the scar formation in the esophagus and the extensive mucosal involvement we concluded the patient had mucous membrane pemphigoid (MMP) rather than bullous pemphigoid. Due to worsening nutritional status a dobhoff tube was placed for nutrition. The patient unfortunately died from cardiac arrest.MMP is a rare, 2 cases per million, auto-immune relapsing and remitting inflammation of the mucosa that leads to chronic subepithelial blistering, mucosal fibrosis and scarring of healed lesions which distinguishes it from BP which usually heals without scar formation. MMP is characterized by inflamed and eroded mucosa but can also have blistering lesions of the skin while BP is characteristic lesion are tense bullae and rarely effects mucous membranes. In a study of 457 cases of MMP only 4% affected the esophageal mucosa. MMP is diagnosed by the gold standard of direct immunofluorescence with linear IgG or IgA or C3 staining along the basement membrane. Goal of treatment is to decrease blister formation and promote healing which is accomplished with glucorticoids, immune-suppressants, like mycophenolate and acid suppression. As mucosal lesions heal and scar formation takes place strictures often form and require esophageal dilation. Disease progression can be chronic and life threatening if it effects mucous membranes of the airways. MMP is associated with an increased risk for malignancy-associated laminin 332 lymphoma.1712_A Figure 1. Esophageal Denuding and Blistering due to Mucous Membrane Pemphigoid.1712_B Figure 2. Esophageal Scarring after Mucous Membrane Pemphigoid.