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Langerhans cell histiocytosis of the temporal bones: a clinical case

DOI: https://doi.org/10.29296/25877305-2022-01-13

Professor Kh. Diab, MD; Corresponding Member of the Russian Academy of Sciences, Professor N.
Daikhes, MD; V. Saidulaev, Candidate of Medical Sciences; Professor A. Yunusov, MD; O. Pashinina, Candidate
of Medical Sciences; A. Mikhalevich, Candidate of Medical Sciences National Medical Research Center of
Otorhinolaryngology, Federal Biomedical Agency of Russia, Moscow

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 25-30% 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.

orphan diseases
Langerhans cell histiocytosis
temporal bone
computed tomography

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