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2018, Number 1

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

Langerhans Cell Histiocitosis: New Molecular and Clinical Concepts

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

Language: Spanish
References: 71
Page: 36-44
PDF size: 293.80 Kb.


ABSTRACT

Langerhans cell histiocitosis is a neoplasm of myeloid origin characterized by a clonal proliferation of cd1a+/cd207+ cells. This proliferation and accumulation may present in any organ; therefore, the disease varies in clinical presentation, ranging from localized involvement to a widely-disseminated life-threating disease. Since cutaneous manifestations often represent the only initial manifestation, they are key to diagnosis. The current classification of Langerhans cell histiocytosis divides it into three groups according to the number and location of organs involved (high-risk multisystemic, low-risk multisystemic and involvement of a single organ or system). This new classification guides on the prognosis of the disease and allows to determine the ideal management of the patient. This article reviews the new concepts in the etiopathogenesis of the disease, describes the recent classification of the histiocytic disorders and remarks the diverse cutaneous and extracutaneous manifestations of the disease.


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