Histiocitosis de células de Langerhans en adultos. Reporte de 2 casos

Escalera, Sérgio; Alfonso, Graciela; Freitas, Josefina; Maymó, Daniela; Vigna, Carolina

doi:10.48057/hematologa.v26i3.475

Cited by 1 publication

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References 8 publications

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“…In contrast to usual skin cells, pathological Langerhans cells appear round, functionally and immunologically immature, surrounded by eosinophiles, macrophages, lymphocytes, and sometimes giant multinucleate cells 5 . Diagnosis is performed by biopsy of the lesion to ascertain presence of Birbeck granules, and immunohistochemical confirmation by the markers CD1a, S100 or CD207.…”

Section: Introductionmentioning

confidence: 90%

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Langerhans cell histiocytosis oral lesions in pediatric patients

H Pavan,

Fernández de Preliasco,

Ienco

et al. 2023

Acta Odontol Latinoam

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Langerhans cell histiocytosis (LCH) is a disease with unknown etiology. It presents as single-system (affecting a single organ or tissue) or as multisystem (with or without risk organ involvement). The oral cavity may be involved or be the site of the first manifestation. Aim: To describe, group, and determine the frequency of oral lesions in pediatric patients with LCH, and to relate these lesions to age and the different disease subtypes. Materials and Method: Clinical and radiographic examinations were used to evaluate 95 patients diagnosed with LCH, aged 0 to 16 years, who were referred to the Department of Comprehensive Pediatric Dentistry at the School of Dentistry, University of Buenos Aires. Clinical histories were prepared and informed consents obtained. Lesions were diagnosed by observation, palpation and biopsies, and grouped according to affected tissues into bone, mucosal, and bone-mucosal. Results: 42.1% presented oral lesions, and in 14.73%, these lesions were the first manifestation of LCH. Ninety percent presented only bone lesions, while the remaining 10% presented bone-mucosal and mucosal lesions. In the single-system subtype, 52.5% presented bone lesions. In the multisystem subtypes (with or without risk organs), all three types of lesions were found. The association between age at which LCH was diagnosed and oral tissue involvement showed that bone-mucosal lesions occur in young children (average age 1.4 years) diagnosed with multisystem LCH. Oral mucosa was only affected in reactivations of the disease. Conclusions: A high frequency of oral lesions was observed, which were sometimes the first manifestation of the disease, most often affecting bone tissue. Dentists can play an active role in the initial diagnosis of the disease.

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Section: Introductionmentioning

confidence: 90%

“…It has been associated to a mutation of the gene BRAF 600 E and activation of ERK 1-3 . In 50 % to 90 % of the cases diagnosed, patients are between less than one year old to 15 years old 4 , with an estimated annual incidence of 2 to 10 cases per million children 5 .…”

Section: Introductionmentioning

confidence: 99%