Abstract
Mastocytosis (M) represents a systemic pathology characterized by increased accumulation and clonal proliferation of mast cells in the skin and/or different organs. Broadly, M is classified into two categories: Cutaneous mastocytosis (CM) and systemic mastocytosis (SM). In children, CM is the most frequent form. Unfortunately, pathogenesis is still unclear. It is thought that genetic factors are involved, but further studies are necessary. As for features of CM, the lesions differ in clinical forms. The most important fact is evaluating a pediatric patient with CM. It must comprise laboratory exams (with baseline dosing of total serum tryptase), a skin biopsy (with a pathological exam and, if the diagnosis is unclear, immunohistochemical tests), and a complete clinical evaluation. It is also defining to distinguish between CM and other diseases with cutaneous involvement. As for the management of CM in children, the first intervention implies eliminating trigger factors. The available cures are oral H1 and/or H2 antihistamines, oral cromolyn sodium, oral methoxypsoralen therapy with long-wave psoralen plus ultraviolet A radiation, potent dermatocorticoid, and calcineurin inhibitors. In children, the prognosis of CM is excellent, especially if the disease’s onset is in the first or second years of life.
Highlights
Mastocytosis (M) represents a systemic pathology characterized by increased accumulation and clonal proliferation of mast cells in the skin and/or different organs
We describe in the following the most common form of Cutaneous mastocytosis (CM) in children
Depending on the type of skin lesions associated with CM in children, the differential diagnosis involves several dermatological pathologies that are detailed in Table 2 [37,38,39,40,41,42,43,44,45,46,47,48]
Summary
Mastocytosis (M) represents a systemic pathology characterized by increased accumulation and clonal proliferation of mast cells in the skin and/or different organs. The real discovery was made by Ellis in 1949 when he found mast cells to infiltrate the skin and extracutaneous organs such as the liver, spleen, bone marrow, and lymph nodes [4]. Cutaneous mastocytosis—with the following forms: Diffuse cutaneous mastocytosis, maculopapular cutaneous mastocytosis, urticaria pigmentosa (UP), and solitary skin mastocytoma;. CM was first described by Sezary and Chauvillon in 1936 [8] It is most common in children, representing 90% of all cases in this age group [9]. Maculopapular cutaneous mastocytosis represents the cutaneous form This has the highest prevalence and includes UP and other nodular-plaque forms. Young children over the age of 15 develop mastocytomas [10]
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