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Hand-Schuller-Christian Disease

Hand-Schuller-Christian Disease Causes

Its cause is not known but some generalization points to xanthomatosis (R. S. Rowland:Xanthomatosis and the reticuloendothelial system. Correlation of an unidentified group of cases described as defects in mambraneous bones, exophthalmos and diabetes insipidus (Christian's syndrome). Archives of Internal Medicine, Chicago, 1928, 42: 611-674.)

Hand-Schuller-Christian Disease Definition

Hand-Schuller-Christian disease is a condition which lipids accumulate in the body and manifest as histiocytic granuloma in bones, particularly in the skull, the skin and viscera and which is often accompanied by hepatosplenomegaly and lymphadenopathy.

Hand-Schuller-Christian Disease Symptoms and Signs

Symptoms include defects of the skull and in the mandible, unusually long bones, pelvis, ribs and spine.

Hand-Schuller-Christian Disease Treatment

Theraphy of Hand-Schuller Christian disease varies according to age and the severity of the syndrome. Generally however, it includes orthopaedic-surgical approach, radiotherapy, chemotherapy and or immunotherapeutic treatment (De Lacharriere O, Ougier E. Histiocytose langerhansienne de l'adulte a expression cutanee. Ann Dermatol Venereol. 1990; 117: 303-310).

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