AN UNUSUAL PRESENTATION OF GRANULOMATOSIS WITH POLYANGIITIS WITH PRIMARY SKIN INVOLVEMENT

Document Type

Conference Proceeding

Publication Date

7-17-2025

Publication Title

J Gen Intern Med

Keywords

autoantibody, myeloblastin, rituximab, steroid, acanthocytosis, adult, autoimmunity, bronchoscopy, case report, clinical article, conference abstract, cryoglobulinemia, diagnosis, drug therapy, hematuria, human, human tissue, kidney function, leukocytoclastic vasculitis, male, microscopy, petechia, pleurisy, purpura, purpuric rash, serology, skin biopsy, vasculitis, Wegener granulomatosis

Abstract

CASE: We present a case of a 60-year-old male who had recently been admitted for pleuritic chest pain and had been found to have a lung mass and multiple nodules with concerns for a malignancy diagnosis. He presented with a new onset bullous, hemorrhagic purpuric and petechiae to his bilateral hands, feet, lips, and nares. Notably, the patient had retiform purpura over the bilateral MCP, DIP, and PIP joints on the dorsal surface of the hand. His workup revealed a C-ANCA titer of 1:80 and proteinase-3 autoantibodies level of 116. Skin biopsy demonstrated bullous and leukocytoclastic vasculitis, leading to a diagnosis of granulomatosis with polyangiitis. Although C4 was notably low, cryoglobulin testing was negative. Bronchoscopy determined the lung mass and nodules to be secondary to GPA. Urine microscopy demonstrated microscopic hematuria with acanthocytes and preserved renal function. Following several days of steroids the patient began to have an improvement in his purpuric lesions. Rituximab infusions were initiated for definitive management. IMPACT/DISCUSSION: This case demonstrates the variation in presentation of GPA and the importance of keeping a broad differential inclusive of autoimmune diseases. The clinical triad of upper respiratory, lung, and kidney involvement was largely present in this case, but most notable were skin findings, which are only present in 34% of GPA cases. Skin findings in this case were markedly widespread in involvement and occurred in a symmetric pattern across the extremities. However, rather than anchoring on specific symptoms, this case benefited from keeping an open mind. Additionally, considering overlapping patterns of disease expression and effectively ruling out other manifestations of autoimmune disease such as cryoglobulinemia was important in this case. Treatment in this case was definitive and effective because it addressed the underlying pathophysiological process of GPA by interrupting autosensitization. CONCLUSION: - As vasculitides have extremely varied presentations, it is important to avoid anchoring on a particular diagnosis and to maintain a broad differential - Regardless of presentation, treatment that addressed the underlying pathophysiology, as confirmed by serology, was effective in addressing the varied manifestations of GPA .

Volume

40

First Page

S130

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