Erdem,A.Y.Özyörük,D.Emir,S.Çakmakçı,S.Ceylan,G.G.Toyran,M.Mısırlıoğlu,E.D.2024-07-052024-07-05202221077-411410.1097/MPH.00000000000021962-s2.0-85123272674https://doi.org/10.1097/MPH.0000000000002196https://hdl.handle.net/20.500.14411/4079Introduction: Mastocytosis is a rare and heterogenous disease, and in children it is generally limited to the skin and tends to regress spontaneously in adolescence. Aim: In this study, demographic, clinical, and laboratory characteristics of pediatric patients with mastocytosis, and also coexisting diseases were investigated. Results: A total of 61 pediatric patients were included in the study. The male-to-female ratio was 2.2, the median age was 2 years (range, 0.25 to 19 y), and the median follow-up period was 2.0 years (range, 0.25 to 19 y). Types of clinical presentation at diagnosis consisted of mainly urticaria pigmentosa (45.9%). Seven patients were further investigated with suspicion of systemic mastocytosis, they were followed up, median of 9 years (range, 2.5 to 16 y), and none of them developed systemic disease. Coexisting allergic diseases were recorded in total 5 patients (8.2%). Three patients had immunoglobulin A deficiency, 1 patient had elevated immunoglobulin E level. A patient developed mature B-cell lymphoma with a heterozygous mutation in c-KIT exon 11. Discussion: Cutaneous mastocytosis in children may present as a complex disease with different clinical signs and symptoms. Standardized clinical criteria and guidelines for the follow-up of children with mastocytosis are required. Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.eninfo:eu-repo/semantics/closedAccessCoexisting diseaseCutaneous mastocytosisPediatricArticleQ4Q3441E227E232PubMed:34001789