Histiocytosis X of the head and neck

This paper reviews retrospectively the otolaryngological manifestations of 48 cases of histiocytosis X treated at The Montreal Children's Hospital. Age incidence in these cases compared with other series; and ther was a female preponderance. If temporal bone lesions plus other skull lesions are included in otolaryngological manifestations of histiocytosis X, then on workup evidence of disease was found in the head and neck in 38 of 48 patients (80 per cent). An ENT problem was the presenting compliant in only some of these. If only temporal bone lesions are included, then 17 of 48 (40 per cent) had head and neck involvement. The prognosis became poorer as the disease progressed.