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.