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Hand-Schüller-Christian disease[hand´shil´er kris´chan]
a chronic, slowly progressive form of multifocal Langerhans cell histiocytosis, characterized by granulomatous lesions with histiocyte proliferation involving mainly the bones and skin; it affects children and young adults. The three classic symptoms are softened areas of the skull and other flat bones, exophthalmos, and diabetes insipidus, although all three symptoms are rarely found in one patient. otitis frequently accompanies the disease. Skin lesions resembling those of seborrheic dermatitis may appear, as may xanthomas. Treatment depends on the symptoms associated with the disease. X-ray therapy is sometimes helpful in treating specific local lesions, and corticosteroids have been used with success in some cases. Complete recovery does occur, but about 40 per cent of the cases end fatally. Called also Hand's disease, Schüller's disease, Schüller-Christian disease, and chronic idiopathic xanthomatosis.
an idiopathic condition, classified as a rare disease by the U.S. National Institutes of Health, in which lipids accumulate in the body and manifest as histiocytic granuloma in bone. It occurs most commonly in children 1 to 3 years of age. Also called Hand-Schüller-Christian disease.