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Hend-Schüller-Krischen syndrome: causes, symptoms, diagnosis, treatment
Last reviewed: 07.07.2025
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Hand-Schüller-Christian syndrome is a clinical variety of histiscitosis X, a granulomatous disease of unknown etiology. The clinical picture is characterized by symptoms of diabetes insipidus, exophthalmos (usually unilateral, less often bilateral) and bone defects - mainly of the skull bones, femurs and vertebrae. Bone defects on X-ray examination are foci of enlightenment resembling a geographical map. Skin symptoms (xanthomatosis, papular exanthema and purpura), painless tooth loss, growth retardation, infantilism, hypercholesterolemia, pathological fractures of tubular bones are characteristic. Over a long period of time, the only clinical manifestation of the disease may be symptoms of diabetes insipidus. Hypogonadism, growth retardation due to growth hormone deficiency and partial or complete hypopituitarism are observed much less often. The course is benign.
Pathogenesis of Hand-Schüller-Christian syndrome. As a result of the formation of histiocytic granulomas with eosinophilic elements in the gray tubercle and other parts of the hypothalamus, the production of hypothalamic releasing factors is disrupted, resulting in panhypopituitarism and the production of antidiuretic hormone with the appearance of symptoms of diabetes insipidus. Exophthalmos appears as a result of bone foci of eosinophilic granulomas in the orbits.
The causes of Hand-Schüller-Christian syndrome are unknown.
Treatment of Hand-Schüller-Christian syndrome. Local forms of the disease are treated by curettage in the area of bone changes. Disseminated forms respond to high doses of glucocorticoids or chemotherapy, mainly using cyclophosphamide, methotrexate. As a rule, under the influence of such treatment, neuroendocrine manifestations of the disease do not fully normalize. It is necessary to try to correct them using drugs used to treat diabetes insipidus, panhypopituitarism.
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