Oral pemphigus vulgaris: A case-based rheumatological and immunological perspectives on a rare autoimmune mucocutaneous disease

Abstract

A 52-year-old female patient was referred for suspected rheumatic or connective tissue disease due to one-year history of recurrent oral ulcers along with recent history of nasal ulcers, erythematous rash, puffy fingers, raised erythrocyte sedimentation rate, raised c-reactive protein, and acute inflammatory exudates on biopsy of buccal mucosal ulcers. Her intra-oral examination revealed ulcerative lesions in the mouth. Her anti-nuclear antibody (ANA) by indirect immunofluorescence showed intensity of +2 and cytoplasmic pattern. The repeat deep biopsy from oral lesions was done in view of multiple recurrent inflammatory oral ulcers, non-supportive ANA test, and bleeding with peeling of mucosal surface from oral ulcers on manipulation (Nikolsky’s sign). The histopathological examination showed acantholysis. Her test for anti-desmoglein3 antibody was strongly positive and was diagnosed with oral pemphigus vulgaris (PV). Most of the times, PV is encountered by a dermatologist, but patients with only oral involvement may end up consulting a physician or rheumatologist as a suspected rheumatic or connective tissue disease due to the underlying inflammatory or autoimmune nature. The present case-based review revisits this rare chronic inflammatory autoimmune mucocutaneous disease and provides a recent review of literature based on rheumatological and immunological perspectives.