Hand-Schüller-Christian disease with repeated recurrence

Report of a case with 23 years, follow-up

Abstract

A case of Hand-Schüller-Christian disease with 3 recurrences during 23 years is reported.
The patient was a 4-year-old girl with bone lesions of the skull and maxilla. The lesions were diagnosed histologically as histiocytosis X on biopsy and were treated successfully with steroid therapy. The disease recurred with diabetes insipidus, exophthalmos, bone lesions of the skull, and growth retardation at the age of 14. At the age of 20, bone lesions of the maxilla and femur were noted. In addition, a bone lesion appeared in the mandible 5 years later. Steroid therapy was effective, but the cessation of therapy or reduction of the dose resulted in recurrence of bone lesions. Thus, maintenance steroid therapy may be needed to control the disease.