Head and neck manifestations of histiocytosis-X in children.

The clinical presentation and course of 100 children aged 6 months to 22 years who had histiocytosis-X and were treated at the Children's Hospital of Philadelphia from 1974 to 1987 were reviewed. Sixty-three percent of patients presented with complaints referable to the head and neck. Lesions of the skull occurred most frequently, followed by cephalic rash, osseous mandibular destruction, enlarged neck nodes, and gingival disruption. Overall, during the course of the disease, 82% of the children with histiocytosis-X developed head and neck manifestations. There were ten deaths in this series, all associated with diffuse or multifocal disease. Interestingly, four of these patients presented with cephalic rash as a harbinger of diffuse and eventually fatal illness. The method of diagnosis and management of histiocytosis-X is discussed.