Otological manifestations of Langerhans cell histiocytosis in a child - An enigma that persists

• Published in: Indian journal of otology 2020-10, Vol.26 (4), p.289-292
• Main Authors: Hong, Chow, Abdullah, Asma, Alias, Azila, Yusoff, Suriyati
• Format: Article
• Language: English
• Subjects: Autoimmune diseases; Histiocytosis; Otology; Pediatrics
• ISSN: 0971-7749; 2249-9520
• DOI: 10.4103/indianjotol.INDIANJOTOL_69_20

Langerhans cell histiocytosis (LCH) is a rare nonmalignant entity commonly seen in the pediatric population which encompasses a variety of clinical presentations. Otological involvement in LCH is uncommon which can be manifested as symptoms similar to otomastoiditis or malignancy, thus diagnosis is often delayed. Herein, we report a 1-year 7-month-old boy presented with unresolving left otorrhea complicated with left postauricular swelling. Otoscopic examination revealed granulation tissue in the left external auditory canal. Initial diagnosis of left otomastoiditis was made until high-resolution contrast-enhanced computed tomography of the temporal bone showed soft-tissue masses occupying bilateral mastoids with bony destruction. Histopathological examination with immunohistochemical staining expressed CD1a cytoplasmic immunoreactivity. The diagnosis of LCH was made. This report serves to highlight such a rare clinical entity which often masquerades as other otological diseases and hence becomes a diagnostic challenge in the pediatric population.