Hand-Schuller-Christian disease is a chronic disorder which is histologically a type of Langerhans cell histiocytosis, with onset between 3 and 5 years.
Granulomatous histiocytic lesions are seen in bone and visceral tissues - skin, lung, lymph nodes and liver. A small proportion of cases may present with diabetes insipidus, cranial lesions and exophthalmos. The disease progresses slowly and may become static.
The most frequent sites for bone involvement are the calvarium, mastoids, mandible and sinuses. A frequent development is chronic otitis media. Abnormal dentition, premature eruption of teeth or four quadrant mouth ulcers may be present. The lips may be swollen and bleeding.
The best treatment is chemotherapy, usually vinblastine and prednisolone, with a good response.
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