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Hand-Schüller-Christian Disease: A Detailed Clinical Survey. Results of Short Term Prednisone Therapy in Ten Patients.

L. V. Avioli, M.D.; M. Z. Lazor, M.D.; H. K. Kashima, M.D.; and J. R. Andrews, M.D.
Ann Intern Med. 1961;54(5):1042. doi:10.7326/0003-4819-54-5-1042_1
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Ten patients with the systemic variety of Hand-Schüller-Christian disease (eosinophilic xanthomatous granulomatosis) have been followed for periods of six to 18 months. Osteolytic lesions were observed in all patients; four presented with diabetes insipidus and three with exophthalmos. Seborrheic dermatitis, chronic otitis media, a non-regenerative anemia, and hepatosplenomegaly were also observed clinically. One case presented with exanthoma disseminata and what appeared to represent hypothalmic involvement, since the disease process was manifested by documented deficiencies in adenocorticotropic and thyroid-stimulating hormones. Bone age as well as urinary gonadotrophin levels were normal in this patient. Osteolytic lesions were biopsied and cultured before and


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