Histiocitosis de células de Langerhans de presentación en etapa neonatal.

NEONATAL PRESENTATION OF LANGERHANS CELL HISTIOCYTOSIS.

LANGERHANS-cell histiocytosis; HARD palate; SYMPTOMS; HISTIOCYTOSIS; DENDRITIC cells

Las histiocitosis son un grupo heterogéneo de enfermedades caracterizadas por la proliferación de células del sistema mononuclear fagocítico (monocitos, macrófagos y células dendríticas) en diferentes tejidos, poco frecuentes, de predominio en la edad pediátrica y con severidad variable. La histiocitosis de células de Langerhans (HCL) es un proceso reactivo de etiología desconocida, con expresión clínica y pronóstico variables. Se presenta un recién nacido a término, sin antecedentes relevantes, quien al nacimiento presenta lesiones cutáneas generalizadas con compromiso palmoplantar y de cavidad oral. Inmunohistoquímica en biopsia de piel compatible con HCL congénita. Se consideró enfermedad unisistémica, compromiso cutáneo y de sitio especial a nivel del paladar duro y recibió quimioterapia de acuerdo con el protocolo para el tratamiento de la histiocitosis de células de Langerhans del Instituto Nacional de Cancerología (INC-2012), vinblastina y prednisolona. Culminó el primer ciclo con remisión completa de las lesiones cutáneas y a nivel de la cavidad oral, sin extensión a otros órganos. [ABSTRACT FROM AUTHOR]

Histiocytosis is a heterogeneous group of diseases characterized by the proliferation of cells of the mononuclear phagocytic system (monocytes, macrophages, and dendritic cells) in different tissues. They are infrequent entities, predominantly occurring in childhood, with variable severity. Langerhans cell histiocytosis (LCH) is a reactive process of unknown etiology with heterogeneous clinical manifestations and variable prognosis determined by the age of onset and organ involvement. We present the case of a term newborn with no relevant family or obstetric history, who presented generalized skin lesions with palmoplantar involvement in the oral cavity from birth. A skin biopsy was performed, with histopathological and immunohistochemical findings compatible with the diagnosis of congenital Langerhans cell histiocytosis. Due to being a unisystemic disease, with skin involvement and special site involvement of the hard palate, according to the classification of the Histiocytosis Society, it was considered a candidate for management with chemotherapy by the HCL-INC-2012 protocol, based on vinblastine and prednisolone. The patient completed the first cycle of the initial treatment, with complete remission of the skin lesions and oral cavity, without compromise of other organs. [ABSTRACT FROM AUTHOR]

Copyright of Revista de la Asociación Colombiana de Dermatología y Cirugía Dermatológica is the property of Asociacion Colombiana de Dermatologia y Cirugia Dermatologica and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)