From Purpura to pulmonary hemorrhage: dermatological clues to systemic vasculitis – a case of granulomatosis with polyangiitis
Naqeeb Ullah, Azra Jabin, Huma Isar, Samreen Javed, Farah Gul, Filza Ajmal, Sundas Safdar, Hanif Ullah Hanfi, Ramin Khan, Adnan Ayub, Samim Noori, Sardar Noman QayyumIntroduction:
Granulomatosis with polyangiitis (GPA) is a rare ANCA-associated vasculitis that can convincingly mimic IgA vasculitis when it presents with palpable purpura and a leukocytoclastic biopsy. Failure to perform direct immunofluorescence (DIF) and ANCA testing at presentation is a recognized cause of diagnostic delay and irreversible organ damage.
Case presentation:
A 47-year-old woman, previously labeled as IgA vasculitis on the basis of a leukocytoclastic skin biopsy without DIF, returned 1 year later with recurrent necrotizing leg ulcers, ENT involvement, and hemoptysis. Strong c-ANCA/PR3 positivity (>100 U/mL), microscopic hematuria, an HRCT pattern of diffuse alveolar hemorrhage, and a repeat skin biopsy demonstrating pauci-immune necrotizing leukocytoclastic vasculitis (DIF negative for IgA) confirmed GPA. Pulse methylprednisolone, followed by oral corticosteroids and IV cyclophosphamide, produced complete reversal of pulmonary hemorrhage within 2 weeks and progressive healing of cutaneous ulcers.
Discussion:
Persistent or recurrent cutaneous vasculitis – particularly necrotizing ulcers in an adult – should prompt ANCA testing and DIF, even when an initial diagnosis of IgA vasculitis seems plausible. Pauci-immune histology and PR3-ANCA reliably distinguish GPA from IgA vasculitis and redirect management toward induction immunosuppression.
Conclusion:
Early dermatologic recognition, repeat biopsy with immunofluorescence at any clinical inflection point, and timely induction therapy can reverse otherwise life-threatening alveolar hemorrhage in GPA.