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Langerhans cell histiocytosis of the temporal bones: a clinical case

DOI: https://doi.org/10.29296/25877305-2022-01-13
Issue: 
1
Year: 
2022

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.

Keywords: 
orphan diseases
Langerhans cell histiocytosis
temporal bone
computed tomography



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