Annals of medicine and surgery (2012) · 2026
From Purpura to pulmonary hemorrhage: dermatological clues to systemic vasculitis - a case of granulomatosis with polyangiitis.
Клинический случайСтатьяНазвание и аннотация: Перевод в очереди
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Ullah Naqeeb, Jabin Azra, Isar Huma, Javed Samreen, Gul Farah, Ajmal Filza, Safdar Sundas, Hanfi Hanif Ullah, Khan Ramin, Ayub Adnan, Noori Samim, Qayyum Sardar Noman
Аннотация
INTRODUCTION: 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.
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.