Histiocytosis X of the head and neck

Histiocytosis X is a general disease class for three less distinctive and overlapping states; namely, eosinophilic granuloma, Hand-Schuller-Christian disease, and Lcttercr-Siwe disease. These conditions span a continuum from an isolated bony or soft tissue lesion to a fulminant, disseminated process. The head and neck are frequent sites of initial presentation for the more serious varieties. We present four cases of histiocytosis X with lesions in the temporal bone, skull, and mandible. Some patients had accompanying scalp lesions as well as other distal bony and soft tissue lesions. Treatment included radiation therapy, chemotherapy, and surgery. A rational discussion of new treatment modalities based on stage of the disease is presented. Utilization of CT scanning in the initial diagnosis and follow-up is demonstrated. Management of troublesome otologic complications is also discussed.