Extensive Vulvar Involvement as the Initial Presentation of Granulomatosis with Polyangiitis in a Young Woman - 20/11/25

Doi : 10.1016/j.jpag.2025.08.005

Dubravka Živanović, MD, PhD ^1,², Vesna Kesić, MD, PhD ³, Marija Malinić, MD ¹, Jelena Vuković, MD ¹, Martina Bosić, MD, PhD ⁴, Vesna Reljić, MD, PhD ^1,^2,^⁎
¹ Clinic of Dermatology and Venereology, University Clinical Center of Serbia, Belgrade, Serbia
² Department of Dermatology and Venereology, University of Belgrade, Faculty of Medicine, Belgrade, Serbia
³ Department of Human Reproduction, University of Belgrade, Faculty of Medicine, Belgrade, Serbia
⁴ Institute of Pathology, University of Belgrade, Faculty of Medicine, Belgrade, Serbia

^⁎Address correspondence to: Vesna Reljić, MD, PhD, Clinic of Dermatology and Venereology, University Clinical Center of Serbia, University of Belgrade – Faculty of Medicine, Pasterova 2, 11000 Belgrade, Serbia; Phone: +381 11 366 2477.Clinic of Dermatology and Venereology, University Clinical Center of SerbiaUniversity of Belgrade – Faculty of MedicinePasterova 2Belgrade11000Serbia

ABSTRACT

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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Key Words : Vulvar granulomatosis with polyangiitis, Cutaneous granulomatosis with polyangiitis, Vasculitis, PR-3 ANCA

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Vol 38 - N° 6

P. 759-761 - décembre 2025 Retour au numéro

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Extensive Vulvar Involvement as the Initial Presentation of Granulomatosis with Polyangiitis in a Young Woman - 20/11/25

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Conclusion

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