Bioptic diagnosis of Wegener's granulomatosis in the absence of vasculitis and granulomas.

We report a case of Wegener's granulomatosis (WG) which differed significantly from the WHO-definition. The patient presented himself with fever and skin lesions which exacerbated after penicillin therapy. The nasal biopsy did not reveal the expected classical vasculitis and granulomas, which were present at autopsy only in the liver and spleen. We emphasize that vasculitis and granulomas may be absent in the biopsy specimen. We stress the institution of chemotherapy with cyclophosphamide, once the clinicopathological impression of WG is probable.