Langerhans cell histiocytosis (LCH). Overview of symptoms of LCH, which may lead the patients to any of these medical specialists.

Adam, Zdeněk; Pour, Luděk; Tomíška, Miroslav; Starý, Karel; Horváth, Teodor; Doubková, Martina; Nebeský, Tomáš; Řehák, Zdeněk; Koukalová, Renata; Krejčí, Marta; Krejčí, Miroslav; Boichuk, Ivanna; Štork, Martin; Ševčı́ková, Sabina; Adamová, Zuzana; Král, Zdeněk

doi:10.36290/vnl.2022.027

Cited by 1 publication

(1 citation statement)

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“…8 ECD is a multisystem disease, with patients displaying extra-axial osteosclerosis, perirenal infiltration, diabetes insipidus, interstitial lung disease, central nervous system (tumoral or degenerative cerebellum) involvement, exophthalmos, 9 atrial pseudotumor and aorta impairments. 4 Ear, nose and throat (ENT) involvement has been little described but the manifestations reported include bilateral sensorineural hearing loss secondary to internal auditory canal infiltration, 10 external auditory canal (EAC) stenosis, 11,12 a condition mimicking rhinoscleroma, 13 nasal mass 11 and maxillary sinus and facial bone sclerosis. 8 Our aim in this study was to calculate the prevalence of sinonasal and ear involvement in a population of ECD patients to describe the various ENT manifestations observed and to investigate the associations between ENT manifestations, the involvement of other organs and BRAF mutations.…”

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confidence: 99%

Sinonasal and ear manifestations of Erdheim‐Chester disease

et al. 2023

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SummaryTo calculate the prevalence of sinonasal and ear involvement in an Erdheim‐Chester disease (ECD) population, to describe the different ear, nose and throat (ENT) manifestations and to study the association between ENT involvement, other organ involvement, and BRAF mutations. We led a retrospective monocentric study in the national referral center for ECD. One hundred and sixty‐two patients with ECD and ENT data were included between January 1, 1980 and December 31, 2020. Ear and nose clinical and radiological findings were noted. We described and studied the prevalence of ENT involvement in ECD population. The association between sinonasal and ear involvement, other organ involvement, and BRAF mutations was calculated. The prevalence of ENT manifestations is around 45%. No clinical rhinologic or otologic signs were specific to ECD. Sinus imaging was abnormal in 70% of cases. A bilateral maxillary sinus frame osteosclerosis was highly specific of ECD. Associations were found between the sinus MRI imaging type and BRAF status, central nervous system involvement, cerebellum involvement and xanthelasma. Sinonasal or ear involvement is frequent in ECD and has specific imaging features for sinuses.Trial registration: #2011‐A00447‐34.

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