Introduction
Pemphigus is a group of potentially life-threatening autoimmune mucocutaneous diseases characterized by epithelial blistering affecting cutaneous and/or mucosal surfaces secondary to IgG antibody synthesis against a desmosome glycoprotein, desmoglein (Dsg), present in keratinocytes. Its estimated incidence is at two new cases/ millions/year. Most are seen in the fifth and sixth decades of life with rare cases seen in children and the elderly.1 Although there are variants within pemphigus, pemphigus vulgaris accounts for 80% of cases. There are two clinical forms of pemphigus vulgaris: mucosal (MPV) and mucocutaneous (MCPV) which patients’ mucosa is affected and over five skin lesions are present.2 The recent study showed that patients with pemphigus vulgaris have oral lesion about 90% with solely oral mucosal lesion around 50%.3 The presentation symptoms include tenderness in the throat, pain on swallowing, hoarseness or dysphonia.2 The authors present a case of pemphigus vulgaris with oropharyngeal and esophageal involvement presenting uniquely with an epigastric pain.