Hand-Schuller-Christian disease


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Noun1.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).
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).
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.