Manifestações Cutâneas nas Mastocitoses: Atualização

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 cutaneousmastocytosis, diffuse cutaneous mastocytosis and cutaneous astocytoma. Telangiectasia macularis eruptiva perstans is no longerconsidered 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.

Case Report and Review of the Literature: Bullous Skin Eruption After the Booster-Dose of Influenza Vaccine in a Pediatric Patient With Polymorphic Maculopapular Cutaneous Mastocytosis

“…Darier's sign, pruritus, and dermographism are usually present, while blistering may occur, especially in children under 3 years old. Serum tryptase levels are usually in the normal range; rarely patients with pronounced lesions can present increased level that usually decreases within 1 or 2 years (8,14). The prognosis is excellent, and usually, lesions resolve by adolescence (2).…”

“…The lesions are usually smaller, round, and similar. In these patients, serum tryptase levels can be persistently increased over time, and the disease tends to persist into adulthood with a higher risk of developing systemic mastocytosis (3,8,14).…”

“…It usually occurs in the form of a single lesion, yellow-brownish, with the frequent formation of pomphoid lesions, the possible formation of bullous skin eruptions, and regional or diffuse flushing after the mechanical stimulation of the lesion. It has a dynamic pattern and can increase by dimensions (but not number) and usually regress by mid-teenage years (14,15). According to Hartmann et al, a maximum of three compatible lesions still should qualify as mastocytoma, while more than three lesions should be considered as attributable to a case of MPCM (3).…”

“…According to Hartmann et al, a maximum of three compatible lesions still should qualify as mastocytoma, while more than three lesions should be considered as attributable to a case of MPCM (3). The diagnosis is based exclusively on clinical manifestations, and serum tryptase levels are usually within the normal range (3,14).…”