A 51-year-old woman presented with a 4-month history of painful ulcers in the mouth and vulva, and painful vegetative plaques at intertriginous sites. Skin biopsies showed squamous hyperplasia and intraepidermal eosinophilic pustulation. Skin direct immunofluorescence (DIF) revealed intercellular deposition of IgG and C3 in the lower part of the epidermis, while serum indirect immunofluorescence (IIF) confirmed the presence of antiepithelial antibodies. The patient was diagnosed with pemphigus vegetans, and successfully treated with dapsone, prednisolone and topical steroids. Although pemphigus vegetans and pyostomatitis-pyodermatitis vegetans can show identical clinical and histological features, the presence or absence of comorbid inflammatory bowel disease, and the results of both skin DIF and serum IIF can be used to distinguish between these two conditions. This case report explores the challenges in making this distinction, and the implications of establishing the correct diagnosis.
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Contributors The case report was prepared by BS, with supervision from SS and AS. The patient’s dermatological condition is managed by SS, and the diagnosis was established in consultation with AS.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.
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