LANGERHANS’ CELL HISTIOCYTOSIS: HEAD AND NECK MANIFESTATIONS IN CHILDREN Muhammad S. Quraishi, FRCS, Alexander W. Blayney, FRCS, David Walker, MRCP, Fin 8. Breatnach, FRCP, and Patrick J. Bradley, FRCS zyxwv Background. Langerhans’ cell histiocytosis (LCH) is an un- common, poorly understood granulomatous disease, char- acterized by the idiopathic proliferation of Langerhan’s cells or their marrow precursors. In 1985, the Philadelphia Work- shop adopted the term “Langerhans’ cell histiocytosis” (LCH) to differentiate it from reactive and neoplastic causes of histiocytosis. Methods. This study includes zyxwvuts 73 pediatric patients diag- nosed with this condition in Dublin, Ireland, and Nottingham, England, during a 34-year period (1959 to 1993). These pa- tients are reviewed with respect to clinical presentation, dif- ficulty with making a histological diagnosis, their manage- ment, and outcome. Resufrs. A total of 49 patients (67%) had head and neck involvement. Bony involvement was the most frequent sign, most frequently located in the skull. There were 11 deaths (15%) in this series, all associated with multisystem disease, and nine of these deaths were in children younger than 2 years of age. Conclusions. The role of otolaryngologists is important in the early and accurate evaluation, staging, and diagnosis From University Hospital (Mr Quraishi. Waiker, and Bradley), Nottingham, England; Temple Street Children’s Hospital (Mr. Blayney). Dublin, Ireland; and Our Lady’s Hospital for Sick Children (Mr Breatnach), Dublin, Ire- land. Presented at the Irish Otolaryngological Society Meeting, Killarney. Ire- land, October 1993. Acknowledgments: We thank Dr. Peter Barbor for allowing us to include his patients in this study. We also thank Ms. Christine Turner and Ms. Dervilla Dempsey for their secretarial assistance. Address reprint requests to Mr Quraishi at the ENT Department, Univer- sity Hospital, Nottingham NG7 2UH, United Kingdom. Accepted for publication October 14. 1994. zyxwvutsrqpo 0 1995 John Wiley & Sons, Inc. of LCH. It may mimic more common diseases, such as otitis externa, acute mastoiditis, skin rash, gingivitis, or cervical lymphadenopathy. Patients with multisystem disease may be so ill at presentation that the head and neck lesions may be overlooked. The current management of LCH has become increasingly conservative, and in the 199Os, fewer cases are given chemotherapy or radiotherapy. The prognosis is very good for single-system disease and poor for multisystem dis- seminated disease with early onset. HEAD & NECK 1995;17:226231 0 1995 John Wiley & Sons, Inc. zyxw Langerhans’ cell histiocytosis is characterized by a proliferation of Langerhans’ cells, regarded as a subpopulation of the mononuclear cell system,’ sharing biological and morphological properties with macrophages. The diseases Letterer-Siwe disease, Hands-Schuller-Christian syndrome, and eosinophilic granuloma were grouped together in 1953 into a single group called histiocytosis zy X. In 1985, the Philadelphia Workshop’ adopted the term “Langerhans’ cell histiocytosis” (LCH) to differentiate it from reactive and neoplastic causes of histiocytosis. This condition (LCH) is classified according to the site(s) of involvement into single-system disease, which may be subdi- vided into unifocal or multifocal or multisystem disease if more than one body function system is involved. HISTORY OF DISEASE Alfred Hand in 18932described a skull lesion in a 3-year-old child who also had exophthalmos, poly- 226 Langerhans’ Cell Histiocytosis HEAD & NECK May/June 1995