DERMATOLOGY: Edited by Albert C. YanPediatric mastocytosisHussain, Sadaf H.a,b Author Information aDepartment of Allergy and Immunology, Boston Children's Hospital bDepartment of Dermatology, Harvard Medical School, Boston, Massachusetts, USA Correspondence to Sadaf H. Hussain, MD, FAAD, Boston Children's Hospital, 300 Longwood Ave, Fegan 6, Boston, MA 02115, USA. Tel: +1 617 355 6117; e-mail: [email protected] Current Opinion in Pediatrics: August 2020 - Volume 32 - Issue 4 - p 531-538 doi: 10.1097/MOP.0000000000000922 Buy Metrics Abstract Purpose of review The current article highlights recent developments in the field of pediatric cutaneous mastocytosis. Mastocytosis is a spectrum of conditions that range from fleetingly benign to aggressively malignant. Through recognizing the natural progression of disease, the role of biomarkers and mutational analysis, treatment and risk of triggers, physicians can confidently stage, counsel and manage patients with pediatric cutaneous mastocytosis. Recent findings Many lesions of cutaneous mastocytosis are chronic with some resolving around the mid-teenage years. KIT mutations are found in the majority of pediatric cutaneous mastocytosis but are not correlated with prognosis. Serum tryptase levels may be elevated in pediatric cutaneous mastocytosis patients without systemic mastocytosis. Pimecrolimus, omalizumab and tyrosine kinase inhibitors are effective treatment options. The low risk of NSAIDs and vaccinations has been characterized and epinephrine autoinjectors are rarely utilized in the pediatric cutaneous mastocytosis patient. Summary Pediatric cutaneous mastocytosis is a heterogeneous disease with good outcome overall. Organomegaly, elevated tryptase levels and the presence of KIT mutation in peripheral blood may aid in the decision to pursue bone marrow biopsy. The armamentarium of treatments has expanded and better understanding of the significance of triggers and vaccination safety allows the clinician to thoughtfully counsel and allay anxiety around pediatric cutaneous mastocytosis. Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.