Oral pemphigus

Definition

Oral pemphigus may exist alone but is more usually a part of pemphigus vulgaris, preceding the appearance of skin lesions by weeks or months and rarely years. The disease begins with blisters (bullae) in the mouth, which are often mistaken for aphthae. The blisters are flaccid and are easily broken, leading to often painful lesions or erosions. Autoantibodies directed against the desmosome component desmoglein 3 (oral epithelial membrane adhesion molecule) cause acantholysis and intraepidermal clefting. Diagnosis can be confirmed by standard histopathology showing intraepidermal blisters due to suprabasal acantholysis and direct and indirect immunofluorescence showing IgG at the cell surface of epithelial cells. The disease activity is roughly correlated with the level of circulating antibodies. Systemic steroids are the initial treatment of choice and can be combined with immunosuppressant drugs. The prognosis of pemphigus has markedly improved over the last decades nevertheless mortality can occur mostly as a consequence of infection.

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