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Actas Dermosifiliogr. 2016 Jan-Feb;107(1):5-14. doi: 10.1016/j.ad.2015.09.007. Epub 2015 Nov 3.

Update on Mastocytosis (Part 1): Pathophysiology, Clinical Features, and Diagnosis.

[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 various organs. The organ most often affected is the skin. Mastocytosis is a relatively rare disorder that affects both sexes equally. It can occur at any age, although it tends to appear in the first decade of life, or later, between the second and fifth decades. Our understanding of the pathophysiology of mastocytosis has improved greatly in recent years, with the discovery that somatic c-kit mutations and aberrant immunophenotypic features have an important role. The clinical manifestations of mastocytosis are diverse, and skin lesions are the key to diagnosis in most patients.

KEYWORDS:

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

PMID:
26546030
DOI:
10.1016/j.ad.2015.09.007
[PubMed - in process]
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