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Updated: Jan 18, 2026

Granulocyte-dependent Autoantibody-induced Skin Blistering
Published on: October 12, 2012
Extensive Vulvar Involvement as the Initial Presentation of Granulomatosis with Polyangiitis in a Young Woman
Dubravka Živanović1, Vesna Kesić2, Marija Malinić3
1Clinic of Dermatology and Venereology, University Clinical Center of Serbia, Belgrade, Serbia; Department of Dermatology and Venereology, University of Belgrade, Faculty of Medicine, Belgrade, Serbia.
Introduction:
Granulomatosis with polyangiitis (GPA) is a rare systemic vasculitis, rarely affecting the genitourinary tract. Vulvar involvement is extremely uncommon and often misdiagnosed.
Case Presentation:
A 21-year-old female patient presented with a 1-month history of necrotic vulvar lesions and skin ulcerations. The markedly elevated PR3-ANCA and histopathological findings supported the diagnosis of GPA. Imaging methods revealed multiple spleen infarctions and pseudo-masses in the kidneys; endocranial MRI showed pansinusitis and otomastoiditis. Initial treatment with systemic corticosteroids and methotrexate was insufficient; however, rituximab achieved significant improvement, resulting in complete healing of both vulvar and skin lesions.
Conclusion:
Vulvar GPA, though rare, may be the initial sign of systemic vasculitis. Multidisciplinary evaluation is important for early diagnosis and effective immunosuppressive therapy.
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