Hand-Schüller-Christian disease

Dr Henry Knipe and Dr Euan Zhang et al.

Hand-Schüller-Christian disease is a clinically intermediate form of a spectrum of histiocytic disorders, which ranges from acute fulminant to chronic indolent disease. It primarily affects children, less often young adults, and rarely older adults.

Hand-Schüller-Christian disease has been described as a multifocal unisystem disease, meaning it involves multiple sites of one organ system, almost always bone 2. However, it is classically associated with the triad of diabetes insipidus, proptosis, and lytic bone lesions. This triad is only seen in about a third of patients.

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Article information

rID: 51172
System: Haematology
Section: Pathology
Synonyms or Alternate Spellings:

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