Bilateral Langerhans Cells Histiocytosis in the Temporal Bone: A case report

Abstract

Langerhans cells histiocytosis (LCH) or histiocytosis-X is a rare group diseases that includes three overlapping diseases of Hand-Schuller-Christian disease (HSC), Letterer-Siwe disease (LS)and Eosinophilic granuloma(EG). It results from clonal proliferation of histiocytes having similar morphology and immunophenotype to Langerhans cells in skin and mucosa Though head and neck manifestation is common, isolated simultaneous bilateral temporal bone Langerhans Cell Histiocytosis is an extremely rare presentation. Having same otological menifestations, high resolution CT scan is advocated for early identification and differentiation of LCH from other common conditions such as mastoidits,otitis externa ,chronic suppurative otitis media. Diagnosis of LCH is confirmed by biopsy and immunohistochemical staining of S-100 protein and or CD1a antigen. Chemotherapy is main mode of treatment.The prognosis is worse when presenting age of children is younger than 2 years. 1. Associate Professor, Department of Otolaryngology and Head-Neck Surgery, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh. 2. Professor, Department of Otolaryngology and Head-Neck Surgery, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh 3. Assistant Registrar, Department of ENT, Shaheed Tajuddin Ahmed Medical College, Gazipur,Bangladesh. 4. Associate Professor, Department of Radiology and Imaging, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh 5. Assistant Professor, Department of Pathology, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh Address of Correspondence: Dr. Kanu Lal Saha, Associate Professor, Otology Division, Department of Otolaryngology and Head-Neck Surgery, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh, Room no.702,BlockC,BSMMU, Email:drklsaha@gmail.com Introduction: Langrhans cells histiocytosis (LCH) or histiocytosis-X is a rare group diseases that includes Hand-Schuller-Christian disease (HSC), Letterer-Siwe disease(LS) and Eosinophilic granuloma(EG). It results from clonal proliferation of histiocytes which has similarity in morphology and immunophenotype to Langerhans cells located in the skin and mucosa. The Writing Group of the Histiocyte Society recommendedthe term LCH in 1987. The incidence of LCH is 5.4 per million. It may present any age but average onset of age is between 1-3 years with a male predominance. Though it may involve any organ head and neck is affected in about 55-73% cases. Temporal bone involvement in LCH is variable ranging from 4-61%. Bilateral