Necrotizing sarcoid granulomatosis with suppurative features.

Necrotizing sarcoid granulomatosis has become a well-defined entity within the spectrum of disorders classified as pulmonary granulomatosis with angiitis. It is characterized clinically by disease generally restricted to the chest, steroid sensitivity, and a good prognosis. Pathologically, confluent granulomas, vasculitis, and bland necrosis are seen. The authors report a patient initially presenting with the typical clinical and pathologic features of necrotizing sarcoid granulomatosis who, on subsequent recurrence, demonstrated a suppurative character to the necrosis. This feature has not been reported previously, and its recognition will allow more cases to be diagnosed correctly. Serum angiotensin converting enzyme (ACE) was not elevated, and ACE could not be demonstrated in tissue from the lung biopsy obtained during recurrence of disease. This further suggests significant differences between this entity and sarcoid.