Langerhans cell histiocytosis

Abstract

Langerhans cell histiocytosis (LCH) is a rare proliferative disorder in which Langerhans's cells accumulate in a variety of organs. LCH affects mostly children and adolescents, involves one or many body systems or tissues such as bone, lung, hypothalamus, posterior pituitary gland, skin, lymph node, liver, and various soft tissues. The clinical features of LCH depend on the extent of involvement. Here in, we report a case of 2-year-old girl presented with fever, swelling in front and behind of right ear and ear discharge. She had anaemia and lytic lesions on skull x-ray and high resolution computed tomography. Biopsy was performed and histopathology and immunohistochemistry fi ndings suggested diagnosis of LCH.

Otologic Manifestations of Langerhans Cell Histiocytosis: A Systematic Review

“…The most common otologic symptom was otorrhea, in 46% of patients in this review, which is consistent with previous studies reporting 45% to 55%. 4,11 Conductive hearing is more typical when 45 1 1 1 Yavas et al 62 1 1 1 1 1 1 S Jeunon et al 27 1 1 1 U Meyer et al 46 1 1 Alexander et al 50 1 1 1 1 1 U Arsovic, Tomanovic, and Bukurov 53 1 Bozdemir et al 26 1 1 1 1 1 1 1C Coleman et al 13 1 1 1 1C Gungadeen, Kullar, and Yates 16 1 1 1 Nishal, Modi, and Balar 41 1 1 1 Robison et al 48 1 1 1 1 Abdel-Aziz et al 7 12 10 4 8 10 C, 2 M Skoulakis et al 22 1 1 1 1 1 1 Xie et al 43 1 1 1 1 1 U Ehrhardt et al 44 1 1 Henderson et al 19 1 1 1 1 S Baser et al 18 1 45 1 1 1 V I 1 Yavas et al 62 1 1 1 V I 1 M A Jeunon et al 27 1 1 1 1 VI 1 Meyer et al 46 1 1 Alexander et al 50 1 1 CU Arsovic, Tomanovic, and Bukurov 53 1 1 MA 1 Bozdemir et al 26 1 1 1 1 1M A Coleman et al 13 1 1 1 V I 1 Gungadeen, Kullar, and Yates 16 1 1 AZ 1 1 CU Nishal, Modi, and Balar 41 Robison et al 48 1 1 1 1 M A 1 MT 1 CU Abdel-Aziz et al 7 12 12 MA 12 Skoulakis et al 22 1 1 1 1 1M T Xie et al 43 1 1 1 V I 1 C U Ehrhardt et al 44 1 19 1 1 1 1U S Baser et al 18 1 hearing loss is present, since otic capsule involvement is rare and the disease usually manifests in the middle ear or external auditory canal. 1,4 Occurrence of sensorineural hearing loss may represent otic capsule involvement.…”