Hand-Schuller-Christian disease


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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.

Hand-Schül·ler-Chris·tian dis·ease

(hand shēl'ĕr kris'chĕn),
the chronic disseminated form of Langerhans cell histiocytosis. The classic triad of signs consists of diabetes insipidus, exophthalmos, and bony lesions composed of histiocytes.

Hand-Schüller-Christian disease

A form of histiocytosis X, characterized by a childhood onset of osteolytic lesions of skull and sella turcica, loss of teeth, chronic draining ears. See Langerhans' cell histiocytosis.

Hand-Schül·ler-Chris·tian dis·ease

(hand shē'ler kris'chen di-zēz')
The chronic disseminated form of Langerhans cell histiocytosis. The classic triad of signs consists of diabetes insipidus, exophthalmos, and bony lesions composed of histiocytes.
Synonym(s): Christian disease (1) , Christian syndrome, Schüller syndrome.

Hand-Schuller-Christian disease

A condition of multiple destructive HISTIOCYTOSIS of bone causing skull defects, pituitary gland disturbances with DIABETES INSIPIDUS, middle ear infection and loss of teeth. (Alfred Hand, American paediatrician, 1868–1949, Artur Schuller, Austrian neurologist, 1874–1958, and Henry Asbury Christian, American physician, 1876–1951)

Christian,

Henry Asbury, U.S. internist, 1876-1951.
Christian disease - (1) Synonym(s): Hand-Schüller-Christian disease; - ; (2) Synonym(s): Weber-Christian disease
Christian syndrome - Synonym(s): Hand-Schüller-Christian disease
Hand-Schüller-Christian disease - see under Hand
Weber-Christian disease - see under Weber, Frederick Parkes

Hand,

Alfred, Jr., U.S. pediatrician, 1868-1949.
Hand-Schüller-Christian disease - the chronic disseminated form of Langerhans cell histiocytosis. Synonym(s): Christian disease (1); Christian syndrome; normal cholesteremic xanthomatosis; Schüller disease; Schüller syndrome

Schüller,

Artur, Austrian neurologist, 1874-1958.
Hand-Schüller-Christian disease - see under Hand
Schüller disease - Synonym(s): Hand-Schüller-Christian disease
Schüller phenomenon - in cases of functional hemiplegia, the patient usually turns to the sound side in walking, but in cases of organic lesion, to the affected side.
Schüller syndrome - Synonym(s): Hand-Schüller-Christian disease

Hand-Schül·ler-Chris·tian dis·ease

(hand shēlĕr krischăn di-zēz)
Chronic disseminated form of Langerhans cell histiocytosis; triad of signs consists of diabetes insipidus, exophthalmos, and bony histiocytic lesions.