Supraclavicular and axillary lymphadenopathy as the initial manifestation in Wegener's granulomatosis.

Wegener's granulomatosis (WG) is a systemic granulomatous vasculitis that typically affects the upper airways, lungs and kidneys. Lymphadenopathy is rare in patients with WG. Here, we present the first case of WG whose initial manifestation was superficial lymphadenopathy (i.e. supraclavicular and axillary lymphadenopathy). One year after the initial presentation, a mass appeared in the lung. Biopsy specimens obtained from supraclavicular lymph nodes and lung demonstrated granulomatous vasculitis. This patient was negative for classic antineutrophil cytoplasmic antibodies (c-ANCA). Treatment with glucocorticoids, cyclophosphamide and trimethoprim-sulfamethoxazole has induced complete remission.