Ms. Quraishi et al., AURAL SYMPTOMS AS PRIMARY PRESENTATION OF LANGERHANS CELL HISTIOCYTOSIS, Clinical otolaryngology and allied sciences, 18(4), 1993, pp. 317-323
Langerhan's cell histiocytosis is an uncommon granulomatous disease, c
haracterized by the idiopathic proliferation of Langerhan's cells or t
heir marrow precursors. It encompasses the diseases previously associa
ted with histiocytosis X-eosinophilic granuloma, Hand-Schuller-Christi
an syndrome and Letterer-Siwe syndrome. A series of 54 patients were d
iagnosed with this condition in Dublin over a 33-year-period (1959-199
2). Twenty-seven patients had aural symptoms, of whom 15 had no other
lesions at the time of presentation. Otorrhoea was the most frequent o
tological symptom, followed by lesions in the temporal bone. LCH may m
imic common aural conditions such as otitis externa, otitis media or a
cute mastoiditis and a high index of suspicion is required to recogniz
e it on clinical presentation. The clinical presentation of multi-foca
l disease may be so dramatic that the otologic findings may be initial
ly overlooked. The mortality rate was 14.8%. Therapeutic regimes inclu
ded no treatment, curettage, chemotherapy, radiotherapy or multi-modal
ity treatment.