Langerhans' cell histiocytosis in the paediatric population: presentation and treatment of head and neck manifestations

Abstract

Langerhans' cell histiocytosis (LCH) is a rare paediatric disease of unknown etiology affecting 1 to 5 children per 1 million each year. It is characterized by the idiopathic proliferation of Langerhans' cells. The clinical spectrum of disease is quite varied, ranging from a solitary eosinophilic granuloma to diffuse multisystem involvement. The head and neck is the most common site of involvement, occurring in approximately 60% of LCH patients. Head and neck manifestations are diverse and include skull and temporal bone lesions, cervical lymphadenopathy, and skin rash. Diagnosis can be difficult as these lesions mimic other common conditions seen by the otolaryngologist, including otitis externa, acute mastoiditis, and gingivitis. A retrospective study was carried out to study our centre's experience with LCH over the last 10 years. Twenty-one patients were diagnosed between January 1990 and December 1999. Patient's age at time of diagnosis ranged from 6 days to 14 years. Fifty-seven percent of patients had localized bony lesions; the remaining 43% had diffuse multisystem disease. The head and neck was also the most commonly involved site in our study, affecting 67% of our patients. Presentation and diagnosis of these lesions are discussed in detail. Treatment, complications, and patient outcomes will also be discussed.