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Noun

Hand-Schuller-Christian disease - inflammatory histiocytosis associated with disturbance of cholesterol metabolism; occurs chiefly in young children and is characterized by cystic defects of the skull and diabetes insipidus

histiocytosis - a blood disease characterized by an abnormal multiplication of macrophages

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References in periodicals archive

LCH consists of a constellation of clinical presentations, which include Letterer-Siwe syndrome, a multisystem disease of young children characterized by cutaneous lesions containing infiltrates of Langerhans cells and a poor prognosis; eosinophilic granuloma, a solitary bone lesion; and Hand-Schuller-Christian disease that manifests as a characteristic triad of cranial bone lesions, diabetes insipidus, and exophthalmos (2).

Primary Langerhans Cell Histiocytosis of the Extrahepatic Bile Duct Occurring in an Adult Patient

Three clinical forms of LCH have been identified ranging from localized LCH (eosinophilic granuloma), chronic recurring LCH (Hand-Schuller-Christian disease), and acute disseminated LCH (Letterer-Siwe disease).

Isolated Langerhans Cell Histiocytosis of Orbit: A Case Report and Review of the Literature

INTRODUCTION: We wish to report a very rare case of hand-schuller-christian disease affecting both the twin male children of 6 years age .The diagnosis was made on the basis of classical clinical features, imaging findings, laboratory and histopathology findings.

Hand-schuller-Christian disease in twins: a very rare case

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