6th July 2023

Pemphigus Vulgaris

A potentially fatal autoimmune blistering disorder, typically presenting between the ages of 30 and 60 years. The condition occurs due to intraepithelial blisters caused by IgG antibodies against adhesion proteins desmoglein 3 and/or desmoglein 1. Painful mucosal erosions which can precede skin involvement by months. However, note that mucosal involvement may be the only manifestation of this disorder in some patients. Skin involvement is characterised by flaccid, easily ruptured blisters that quickly evolve into itchy and painful erosions and crusting.

Pemphigus vulgaris

Image Source: Wikimedia Commons (2018). Pemphgoid vulgaris, Licensed under a creative commons licence. Available for free at: https://commons.wikimedia.org/wiki/File:Pemphgoid_vulgaris.jpg (Accessed on: 10 March 2022).

Skin biopsy is sent for histology and direct immunofluorescence. Diagnosis is confirmed on viewing intraepidermal deposition of IgG antibodies and suprabasal epidermal acantholysis. Serum antibody levels can also be taken.

Management involves high dose corticosteroids for disease control followed by a step-down regime. Other immunosuppressive agents such as azathioprine and cyclophosphamide are used as adjuvant or second-line.

Table 8: Variants of pemphigus