Langerhans cell histiocytosis of the temporal bones: a clinical case

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Abstract

Langerhans cell histiocytosis (LCH) is a rare disease with an incidence of 1 to 9 cases per 1,000,000 population per year. From 75 to 90% of cases of the disease occur in childhood. The etiology of the disease is unknown. Pathogenetically, the disease is characterized by a clonal proliferation of Langerhans cells - the lymphoid dendritic cells originating from the bone marrow have an antigen-presenting function outside the dermis. The temporal bones are affected in 2530% of cases. The disease is more common in males. The peak incidence occurs in the first 3 years of life. The disease can be asymptomatic for a long time or be masked as other common middle ear pathologies (chronic suppurative otitis media, mastoiditis, and otitis externa). The article describes a clinical case of temporal bone LCH. The feature of the clinical case in the recurrent course of the disease is that it is masked as acute mastoiditis.

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About the authors

Kh. M.-A Diab

National Medical Research Center of Otorhinolaryngology, Federal Biomedical Agency of Russia

Email: sultan070487@mail.ru

Professor

Russian Federation,

N. A Daikhes

National Medical Research Center of Otorhinolaryngology, Federal Biomedical Agency of Russia

Email: sultan070487@mail.ru

Corresponding Member of the Russian Academy of Sciences, Professor

Russian Federation,

V. A Saidulaev

National Medical Research Center of Otorhinolaryngology, Federal Biomedical Agency of Russia

Email: sultan070487@mail.ru

Candidate of Medical Sciences

Russian Federation,

A. S Yunusov

National Medical Research Center of Otorhinolaryngology, Federal Biomedical Agency of Russia

Email: sultan070487@mail.ru

Professor

Russian Federation,

O. A Pashinina

National Medical Research Center of Otorhinolaryngology, Federal Biomedical Agency of Russia

Email: sultan070487@mail.ru

Candidate of Medical Sciences

Russian Federation,

A. E Mikhalevich

National Medical Research Center of Otorhinolaryngology, Federal Biomedical Agency of Russia

Author for correspondence.
Email: sultan070487@mail.ru

Candidate of Medical Sciences

Russian Federation,

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Supplementary files

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2. Fig. 1. Computed tomography of temporal bone, axial plane; arrow shows area of bony destruction

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3. Fig. 2. Histological view of removed pathological tissue; arrows shows Langerhans cells.

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