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2018, Número 1

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Dermatología Cosmética, Médica y Quirúrgica 2018; 16 (1)


Histiocitosis de células de Langerhans: nuevos conceptos moleculares y clínicos

Morán VE, Durán MC, Orozco CL, Palacios LC, Sáez-de-Ocáriz M, García RMT

Idioma: Español
Referencias bibliográficas: 71
Paginas: 36-44
Archivo PDF: 293.80 Kb.


RESUMEN

La histiocitosis de células de Langerhans es una neoplasia de origen mieloide caracterizada por la proliferación clonal de células cd1a+/cd207+; esta proliferación y acumulación puede ocurrir en cualquier órgano, por lo que la forma de presentación es muy variada, desde formas localizadas hasta cuadros diseminados potencialmente letales. Dado que las manifestaciones cutáneas con frecuencia representan el único dato inicial, son clave para el diagnóstico de esta enfermedad. La actual clasificación de la histiocitosis de células de Langerhans la divide en tres grupos, dependiendo del número de lesiones y la localización de las mismas (afección multisistémica de alto riesgo, multisistémica de bajo riesgo y afección de un solo órgano o sistema). Esta nueva clasificación orienta sobre el pronóstico de la enfermedad y permite normar la conducta terapéutica a seguir. En este artículo se presenta una revisión acerca de los nuevos conceptos en la etiopatogenia de la enfermedad, se describe la reciente clasificación de los trastornos histiocíticos y se mencionan las diversas manifestaciones cutáneas y extracutáneas de la enfermedad.


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