The clinical course of cutaneous mastocytosis in a prospective group of 163 children according to electronic parent survey

Abstract

Introduction. Mastocytosis occurs due to clonal mast cells proliferation and may have different clinical course. As the disease only rarely occurs in pediatric population there are very few clinical observations describing its symptoms, prognosis and therapy response in pediatric cohorts.Aim of the study — characterization of gender, sympthoms, prognosis and therapy in children with mastocytosis using data obtained by electronic parent survey.Methods. The study data was collected from November 2014 till August 2020 from parents referring for consultation of hematologist via “Vkontakte” social network. All parents completed two surveys. The first one was performed right after the referral, the second one was performed from May 2020 to August 2020. The data on age at symptoms onset, family history, symptoms, rash distribution, serum tryptase concentrations, sunlight sensitivity and clinical course was obtained and analyzed.Results. The data on 163 children was obtained, 86 (52.7 %) of them were boys and 77(47.3 %) were girls. The median age was 6 (0.5—22) years. At the end of survey the median observation time was 61.5 (2—276) months. The most common mastocytosis clinical variant was urticaria pigmentosa (n = 129; 79.1 %), mastocytoma in 18 (11 %) and in 15 (9.9 %) an unknown variant was observed. The diagnosis was based on skin biopsy in 19 patients (11.6 %). Characteristic symptoms were seen in 123 (75.4 %) children. The main complaints were skin reaction triggered by various factors (n = 89; 72.3 %) and itching (n = 78; 63.4 %). Most patients took antihistamines (n = 64; 61 %) and ketotifen (n = 22; 21 %). The second survey was conducted in 139 (85.2 %) pts. At the time of survey symptoms progression was seen in 4 (2.9 %) patients, while in 42 (30.2 %) cases stabilization, and in 93 (66.9 %) cases improvement were observed.Conclusion. The symptoms onset mostly occurs in the first year of life. Most frequents symptoms are itching and rash in response to various triggers. Antihistamine drugs led to symptoms mitigation. The clinical course is benign in most children.