Aural symptoms as primary presentation of Langerhan's cell histiocytosis

Abstract

Langerhan's cell histiocytosis is an uncommon granulomatous disease, characterized by the idiopathic proliferation of Langerhan's cells or their marrow precursors. It encompasses the diseases previously associated with histiocytosis X-eosinophilic granuloma, Hand-Schuller-Christian syndrome and Letterer-Siwe syndrome. A series of 54 patients were diagnosed with this condition in Dublin over a 33-year-period (1959-1992). Twenty-seven patients had aural symptoms, of whom 15 had no other lesions at the time of presentation. Otorrhoea was the most frequent otological symptom, followed by lesions in the temporal bone. LCH may mimic common aural conditions such as otitis externa, otitis media or acute mastoiditis and a high index of suspicion is required to recognize it on clinical presentation. The clinical presentation of multi-focal disease may be so dramatic that the otologic findings may be initially overlooked. The mortality rate was 14.8%. Therapeutic regimes included no treatment, curettage, chemotherapy, radiotherapy or multi-modality treatment.