Cutaneous Manifestations in Mastocytosis: Update

Authors

  • Sandra Ferreira Serviço de Dermatologia. Hospital de Santo António. Centro Hospitalar e Universitário do Porto. Porto.
  • Iolanda Fernandes Serviço de Dermatologia. Hospital de Santo António. Centro Hospitalar e Universitário do Porto. Porto. Consulta Multidisciplinar de Linfomas Cutâneos e Mastocitoses. Hospital de Santo António. Centro Hospitalar e Universitário do Porto. Porto. Unidade Multidisciplinar de Investigação Biomédica. Instituto de Ciências Biomédicas Abel Salazar. Porto.
  • Renata Cabral Consulta Multidisciplinar de Linfomas Cutâneos e Mastocitoses. Hospital de Santo António. Centro Hospitalar e Universitário do Porto. Porto. Unidade Multidisciplinar de Investigação Biomédica. Instituto de Ciências Biomédicas Abel Salazar. Porto. Serviço de Hematologia. Hospital de Santo António. Centro Hospitalar e Universitário do Porto. Porto.
  • Susana Machado Serviço de Dermatologia. Hospital de Santo António. Centro Hospitalar e Universitário do Porto. Porto.
  • Margarida Lima Consulta Multidisciplinar de Linfomas Cutâneos e Mastocitoses. Hospital de Santo António. Centro Hospitalar e Universitário do Porto. Porto. Unidade Multidisciplinar de Investigação Biomédica. Instituto de Ciências Biomédicas Abel Salazar. Porto. Serviço de Hematologia. Hospital de Santo António. Centro Hospitalar e Universitário do Porto. Porto.
  • Manuela Selores Serviço de Dermatologia. Hospital de Santo António. Centro Hospitalar e Universitário do Porto. Porto. Unidade de Investigação em Dermatologia. Hospital de Santo António. Centro Hospitalar e Universitário do Porto. Porto.

DOI:

https://doi.org/10.20344/amp.12189

Keywords:

Mast-Cell Sarcoma, Mastocytosis, Cutaneous, Systemic, Skin Diseases

Abstract

Introduction: Mastocytosis is characterized by the clonal expansion of morphological and immunophenotypically abnormal mast cells in different organs. The skin is the most frequently affected tissue. Virtually all children and more than 80% of adult patients with mastocytosis show cutaneous lesions.
Material and Methods: The present article describes the symptoms and signs in cutaneous mastocytosis, based on the review of recently published international consensus guidelines.
Discussion: According to the 2016 World Health Organization classification, mastocytosis can be divided in cutaneous mastocytosis, systemic mastocytosis and mast cell sarcoma. Cutaneous mastocytosis is subclassified in three subtypes: maculopapular cutaneous
mastocytosis, diffuse cutaneous mastocytosis and cutaneous  astocytoma. Telangiectasia macularis eruptiva perstans is no longer
considered a distinct entity.
Conclusion: Based on the age of onset, cutaneous manifestations of mastocytosis can be variable. The classification of cutaneous mastocytosis has recently been updated. Typically, in patients with childhood-onset mastocytosis, the disease occurs as cutaneous mastocytosis and shows spontaneous resolution around puberty. In contrast, adult patients, despite having also cutaneous lesions, often show systemic involvement and the course of the disease is usually chronic.

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Published

2020-04-01

How to Cite

1.
Ferreira S, Fernandes I, Cabral R, Machado S, Lima M, Selores M. Cutaneous Manifestations in Mastocytosis: Update. Acta Med Port [Internet]. 2020 Apr. 1 [cited 2024 May 25];33(4):275-81. Available from: https://actamedicaportuguesa.com/revista/index.php/amp/article/view/12189

Issue

Section

Review Articles