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Actas Dermosifiliogr. 2016 Jan-Feb;107(1):15-22. doi: 10.1016/j.ad.2015.09.009. Epub 2015 Oct 30.

Update on Mastocytosis (Part 2): Categories, Prognosis, and Treatment.

[Article in English, Spanish]

Author information

  • 1Servicio de Dermatología, Complejo Hospitalario Universitario, Albacete, España. Electronic address: jmazana8@gmail.com.
  • 2Servicio de Dermatología, Hospital del Niño Jesús, Madrid, España.
  • 3Instituto de Estudios de Mastocitosis de Castilla La Mancha, Hospital Virgen del Valle, Toledo, España.

Abstract

Mastocytosis is a term used to describe a heterogeneous group of disorders characterized by clonal proliferation of mast cells in different organs. The organ most often affected is the skin. The World Health Organization classifies cutaneous mastocytosis into mastocytoma, maculopapular cutaneous mastocytosis, and diffuse mastocytosis. The systemic variants in this classification are as follows: indolent systemic mastocytosis (SM), aggressive SM, SM with an associated clonal hematological non-mast cell lineage disease, mast cell leukemia, mast cell sarcoma, and extracutaneous mastocytoma. The two latest systemic variants are rare. Although the course of disease is unpredictable in children, lesions generally resolve by early adulthood. In adults, however, the disease tends to persist. The goal of treatment should be to control clinical manifestations caused by the release of mast cell mediators and, in more aggressive forms of the disease, to reduce mast cell burden.

KEYWORDS:

Cutaneous mastocytosis; Mastocitoma; Mastocitosis; Mastocitosis cutánea; Mastocitosis sistémica; Mastocytoma; Mastocytosis; Systemic mastocytosis; Triptasas; Tryptases; Urticaria pigmentosa

PMID:
26525106
DOI:
10.1016/j.ad.2015.09.009
[PubMed - in process]
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