Pemphigus vulgaris-a blistering clinical enigma: case report

Upper aerodigestive tract involvement in pemphigus requires early multidisciplinary evaluation because there can be involvement in the oral cavity, pharynx, larynx and glottis and thus to upper airway as well. Pemphigus vulgaris (PV) is an autoimmune blistering disorder characterized by painful mucocutaneous vesicles and bullae that rupture to form severe erosions. The disease results in intraepithelial blister formation due to immunoglobulin G autoantibodies targeting desmoglein-3(DSG3), and sometimes desmoglein 1(DSG1). Oral lesions are the most common manifestation and serve as the initial sign in 80-90% of cases. Early diagnosis and management of oral involvement are essential to prevent skin lesions and improve the likelihood of achieving early remission. Corticosteroids remain the cornerstone of therapy.

A 35-year-old South Asian male of Indian origin, belonging to the Dravidian ethnic group, presented with a one-month history of multiple ulcers in the oral cavity marked by intermittent exacerbations and remissions. Extra-oral examination revealed multiple blisters in chest and back of the body. Intra-oral examination revealed multiple oral ulcers and an exfoliative cytology revealed scattered exfoliated epithelial cells and clumps of rounded acantholytic cells resembling Tzanck cells. Oral incisional biopsy was done for histopathological and immunological investigations and a definitive diagnosis of Pemphigus Vulgaris was made .

This case highlights the importance of thorough clinical evaluation, careful histopathological and immunofluorescence studies to distinguish PV from a broad range of mucocutaneous blistering disorders. Despite limitations in therapeutic availability, appropriate use of systemic corticosteroids, supportive care, and consideration of steroid-sparing agents are critical components of treatment. Multidisciplinary collaboration is essential, especially in cases with upper aerodigestive tract involvement.