Otic Langerhans' Cell Histiocytosis in an Adult: A Case Report and Review of the Literature

Objective. To present a case of otic Langerhans' cell histiocytosis in an adult. Also included the diagnosis and management of the condition and a review of the relevant literature. Case Report. We report a case of a 41-year-old man with a history of persistent unilateral ear discharge associated with an aural polyp. Radiological imaging showed bony lesions of the skull and a soft-tissue mass within the middle ear. Histological analysis of the polyp demonstrated Langerhans' cell histiocytosis. His otological symptoms were completely resolved with the systemic therapy. Conclusions. Otic Langerhans' cell histiocytosis can present in adults. Persistent ear symptoms along with evidence of soft-tissue masses within the ear and bony lesions of the skull or elsewhere should prompt the otolaryngologists to include Langerhans' cell histiocytosis in their differential diagnosis. Management should be with systemic therapy rather than local surgical treatment. 1. Introduction Langerhans’ cell histiocytosis (LCH) is a rare disease that is characterised by the idiopathic proliferation of abnormal histiocytes, either in one organ or as part of a systemic disease [1–3]. LCH is predominantly known to be a disease affecting children and is rare in adults. LCH has been reported in paediatric studies to involve the ear and temporal bones in 14% to 61% of cases [4]. However, only a few cases of ear involvement have been reported in adults. This report describes a case of LCH affecting the ear in a 41-year-old patients and it is our recommendation that nonsurgical management is preferable. 2. Case Report A 41-year-old self-employed brewery engineer was referred to the ENT department with a four-week history of persistent left-sided mucopurulent ear discharge, despite topical antibiotic therapy including gentamicin. He did not have any other otological symptoms, nor any rhinological or upper aerodigestive tract symptoms. His hearing was within normal limits. He was a smoker of 30 cigarettes per day, had a past medical history of hypertension, and had been recently diagnosed with Langerhans’ cell histiocytosis involving the skull, for which he was under the care of Haematology. His medication list included azathioprine for LCH, antihypertensives, and a statin. On examination, his left external auditory canal was very oedematous and was filled with an inflammatory polyp. Cranial nerve examination was unremarkable. He had a PET-CT as part of his LCH workup, which showed increased fludeoxyglucose (FDG) uptake in the frontal bone, left parietal bone, right occipital bone, and