Langerhans Cell Histiocytosis of the Temporal Bone: A Case Report

Abstract

Langerhans cell histiocytosis (LCH) is an uncommon disease, predominantly affecting the pediatric population, and is uncommon in adults. It is characterized by clonal proli- feration of immature dendritic cells in one or more organs. Temporal bone involvement, reported in 15-30% of cranial LCH cases, often mimic common infectious conditions such as otitis or mastoiditis, frequently resulting in delayed diagnosis. This report presents the case of a 22-year-old woman who presented with persistent, treatment-refractory right otalgia, initially managed as acute otitis externa with topical and systemic antibiotic therapy. The clinical course progressed with otorrhea, vestibular symptoms, associated with mandibular pain. Physical examination revealed significant edema of the posterior wall of the external auditory canal, without tympanic membrane involvement. Compu- ted tomography revealed a mastoid osteolytic lesion, while magnetic resonance imaging showed aggressive lesions in the petrous bone and mandible. The diagnosis of LCH was confirmed by biopsy. Positron Emission Tomography-Computed Tomography revealed systemic dissemination with pulmonary, breast, and osseous involvement, confirming multisystemic disease. This case emphasizes the importance of including LCH in the di- fferential diagnosis of otologic pathologies with a torpid course, highlighting the key role of the otolaryngologist in early suspicion and exhaustive systemic evaluation for a timely diagnostic and therapeutic approach.