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.
- 1. Satter EK, High WA. Langerhans cell histiocytosis: a review of the current recommendations of the Histiocyte Society. Pediatr Dermatol. 2008 May-Jun;25(3):291-5.
- 2. Jaffe ES, Organization WH. Pathology and genetics of tumours of haematopoietic and lymphoid tissues. Iarc. (2001) ISBN:9283224116.