Sandra Ferreira1, Iolanda Fernandes2, Renata Cabral3, Susana Machado1, Margarida Lima3, Manuela Selores4. 1. Serviço de Dermatologia. Hospital de Santo António. Centro Hospitalar e Universitário do Porto. Porto. Portugal. 2. 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. Portugal. 3. 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. Portugal. 4. 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. Portugal.
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.
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.