• Sandra Ferreira, Iolanda Fernandes, Renata Cabral, Susana Machado, Margarida Lima, and Manuela Selores.
• Serviço de Dermatologia. Hospital de Santo António. Centro Hospitalar e Universitário do Porto. Porto. Portugal.
• Acta Medica Port. 2020 Apr 1; 33 (4): 275-281.

IntroductionMastocytosis 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 MethodsThe present article describes the symptoms and signs in cutaneous mastocytosis, based on the review of recently published international consensus guidelines.DiscussionAccording 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.ConclusionBased 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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