Cutaneous Mastocytosis in Adults and Children: New Classification and Prognostic Factors

Immunol Allergy Clin North Am. 2018 Aug;38(3):351-363. doi: 10.1016/j.iac.2018.04.001. Epub 2018 May 17.

Abstract

The skin is one of the most frequent tissues affected in patients with mastocytosis, but cutaneous lesions are highly heterogeneous in shape, size, color, number, localization, and distribution. The World Health Organization recognizes 3 subtypes of cutaneous mastocytosis (CM): maculopapular CM (MPCM), diffuse CM, and mastocytoma of skin. An international task force of experts in mastocytosis has recently proposed subdividing MPCM into monomorphic and polymorphic, which could predict the duration of the disease in children. More research is warranted to develop an improved classification of CM that ideally should incorporate robust factors with prognostic impact on disease behavior.

Keywords: Classification; Cutaneous mastocytosis; Mast cell; Mastocytosis; Prognosis; Urticaria pigmentosa; WHO.

Publication types

  • Review

MeSH terms

  • Adult
  • Child
  • Humans
  • Mast Cells / physiology*
  • Mastocytosis, Cutaneous / diagnosis*
  • Mastocytosis, Cutaneous / genetics
  • Mastocytosis, Cutaneous / immunology
  • Mutation / genetics
  • Prognosis
  • Proto-Oncogene Proteins c-kit / genetics
  • Risk
  • Skin / pathology*
  • World Health Organization

Substances

  • Proto-Oncogene Proteins c-kit