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Hand - Schuller - Christian Disease

definition

Chronic variant of Langerhans cell histiocytosis, a reactive condition of unknown aetiology in which cells with the phenotype of Langerhans cells accumulate in various tissues and cause damage to them. Classically, this syndrome describes a triad of osteolytic defects, diabetes insipidus, and exophthalmos, which occurs in only 10 per cent of chronic differentiated cases. The lungs and pleura are affected in 30 per cent of patients. Chronic otitis media secondary to Langerhans cell infiltration of the mastoid is common. Skin lesions present as coalescing, scaling or crusted, brown to flesh-coloured papules, and granulomatous infiltrates with ulceration are also found in the axillary or anogenital areas.

synonyms

Hand - Schuller - Christian Disease

UMLS

Cholesterol thesaurismosis, Chronic differentiated progressive histiocytosis, Chronic disseminated histiocytosis X, Chronic histiocytosis X, Chronic idiopathic xanthomatosis, Disease, Hand-Schuller-Christian, Generalized histiocytosis of bones, Hand - Schuller - Christian disease, Hand-Schuller-Christian dis., Hand-Schuller-Christian disease, Histiocytosis chronic, X, Histiocytosis, X chronic, Lipid granulomatosis, Schuller - Christian syndrome, Syndrome, Schuller-Christian, Systemic reticuloendothelial granuloma

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