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Similar but different: distinguishing between pemphigus vegetans and pyostomatitis-pyodermatitis vegetans
Brendan Stagg1, Anna Simpson2, Shireen Sidhu3
1SA Health, Adelaide, South Australia, Australia bmj.stagg@gmail.com.
BMJ Case Reports
|April 21, 2021
Summary
This case report details a patient diagnosed with pemphigus vegetans, a rare autoimmune blistering disease. Effective treatment involved dapsone, prednisolone, and topical steroids, highlighting diagnostic challenges.
Area of Science:
- Dermatology
- Autoimmunology
Background:
- Pemphigus vegetans is a rare autoimmune blistering disease.
- Distinguishing it from pyostomatitis-pyodermatitis vegetans can be challenging due to similar clinical and histological features.
Observation:
- A 51-year-old woman presented with painful oral and vulvar ulcers and vegetative plaques.
- Skin biopsies revealed squamous hyperplasia and intraepidermal eosinophilic pustulation.
- Direct and indirect immunofluorescence confirmed antiepithelial antibodies.
Findings:
- The patient was diagnosed with pemphigus vegetans.
- Successful treatment was achieved with dapsone, prednisolone, and topical steroids.
Implications:
- Accurate diagnosis is crucial, differentiating pemphigus vegetans from pyostomatitis-pyodermatitis vegetans.
- Diagnostic tools include assessing for inflammatory bowel disease and utilizing direct/indirect immunofluorescence.
- This case highlights diagnostic challenges and the importance of correct diagnosis for effective management.
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