A rapid unfavorable outcome of Wegener's granulomatosis in early childhood.

Wegener's Granulomatosis was suspected in a 27-month-old female with a nodular, necrotizing lesion of the nose, diffuse subcutaneous nodules, and erythematous desquamation of the entire body. From 20 months of age on she had a purulent nasal discharge, recurrent infections of the upper and lower respiratory tract, a Coombs positive anemia, and enlargement of the spleen and liver. Treatment with azathioprine and corticosteroids produced transient improvement but three months later a dramatic relapse occurred. Cyclophosphamide was substituted for azathioprine but 10 days later the patient died and the autopsy confirmed the diagnosis of Wegener's Granulomatosis. The early age of onset of the disease may explain the unfavorable outcome, despite treatment with cytotoxic agents.