Lymphomatoid granulomatosis is one of a group of pulmonary disorders characterized by necrotizing, aseptic granulomas, usually with angiitis. Multisystem dissemination is common especially to the skin and central nervous system. The diagnosis can be established only by histologic examination. Lymphomatous transformation apparently occurs in 10–20%. Treatment is generally unsatisfactory, although corticosteroids appear to be indicated initially. We present 4 patients, 3 of whom died relatively rapidly with progressive pulmonary lesions despite therapy with corticosteroids and cytotoxic agents. 1 of these patients developed a lymphoma. The fourth recovered after local excision. Pathologic diagnosis, classification, clinical and radiographic features and therapy are discussed.

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