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

Bishnu Pada Dey,Bishwajit Bhowmik,Kanu Lal Saha,Md Abul Hasnat Joarder,Mohammad Anamul Haque

doi:10.3329/bjo.v26i1.47956

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. Bangladesh J Otorhinolaryngol; April 2020; 26(1): 68-72

Highlights

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.[1,2]
Being a rare disease, LCH is of less Otolaryngological interest.Though head and neck presentations menifestations are common with multisystem involvement, isolated temporal bone involvement is very rare.[9,10]
The early symptoms of LCH involving temporal bone are ear discharge occasionally mixed with blood, otalgia, hearing impairment, dizziness and postauricular swelling.The commonest local sign include granulation tissue or aural polyp in the external auditory canal

Summary

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). Bilateral simultaneous presentation of LCH in temporal bone is a rare entity. Before attending our institution she visited in another ENT center where biopsy was taken from the external auditory canal swelling which histopatology revealed inflammatory tissue. She was anaemic but had no other systemic illness like lymphadenopathy, hepatosplenomegaly, exopthalmous, diabetes insipidus. Computed tomography (CT) scan showed bilateral destruction of cortical temporal bone with erosion of tegmen and sigmoid sinus with soft tissue density in both middle ear and external auditory canal but inner ear appeared normal.

Discussion

Conclusion