{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,29]],"date-time":"2026-10-29T19:15:41Z","timestamp":1793301341137,"version":"4.5.0"},"reference-count":172,"publisher":"MDPI AG","issue":"5","license":[{"start":{"date-parts":[[2021,3,4]],"date-time":"2021-03-04T00:00:00Z","timestamp":1614816000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Polish Ministry of Science and Higher Education","award":["ST 02-0066\/07\/253"],"award-info":[{"award-number":["ST 02-0066\/07\/253"]}]},{"name":"Austrian Science Fund (FWF)","award":["P32470-B and F4704-B20"],"award-info":[{"award-number":["P32470-B and F4704-B20"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Pediatric mastocytosis is a heterogeneous disease characterized by accumulation of mast cells in the skin and less frequently in other organs. Somatic or germline mutations in the KIT proto-oncogene are detected in most patients. Cutaneous mastocytosis is the most common form of the disease in children. In the majority of cases, skin lesions regress spontaneously around puberty. However, in few patients, mastocytosis is not a self-limiting disease, but persists into adulthood and can show signs of systemic involvement, especially when skin lesions are small-sized and monomorphic. Children with mastocytosis often suffer from mast cell mediator-related symptoms. Severe hypersensitivity reactions can also occur, mostly in patients with extensive skin lesions and blistering. In a substantial number of these cases, the triggering factor of anaphylaxis remains unidentified. Management of pediatric mastocytosis is mainly based on strict avoidance of triggers, treatment with H1 and H2 histamine receptor blockers, and equipment of patients and their families with epinephrine auto-injectors for use in severe anaphylactic reactions. Advanced systemic mastocytosis occurs occasionally. All children with mastocytosis require follow-up examinations. A bone marrow investigation is performed when advanced systemic mastocytosis is suspected and has an impact on therapy or when cutaneous disease persists into adulthood.<\/jats:p>","DOI":"10.3390\/ijms22052586","type":"journal-article","created":{"date-parts":[[2021,3,5]],"date-time":"2021-03-05T00:39:07Z","timestamp":1614904747000},"page":"2586","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":68,"title":["Molecular Background, Clinical Features and Management of Pediatric Mastocytosis: Status 2021"],"prefix":"10.3390","volume":"22","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6967-1393","authenticated-orcid":false,"given":"Magdalena","family":"Lange","sequence":"first","affiliation":[{"name":"Department of Dermatology, Venereology and Allergology, Medical University of Gda\u0144sk, 80-211 Gda\u0144sk, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4595-8226","authenticated-orcid":false,"given":"Karin","family":"Hartmann","sequence":"additional","affiliation":[{"name":"Division of Allergy, Department of Dermatology, University Hospital Basel and University of Basel, 4031 Basel, Switzerland"},{"name":"Department of Biomedicine, University Hospital Basel and University of Basel, 4031 Basel, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Melody C.","family":"Carter","sequence":"additional","affiliation":[{"name":"Mast Cell Biology Section, Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4532-1644","authenticated-orcid":false,"given":"Frank","family":"Siebenhaar","sequence":"additional","affiliation":[{"name":"Dermatological Allergology, Department of Dermatology and Allergy, Charit\u00e9\u2014Universit\u00e4tsmedizin Berlin, Corporate Member of Freie Universit\u00e4t Berlin, Humboldt-Universit\u00e4t zu Berlin, Berlin Institute of Health, 10117 Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ivan","family":"Alvarez-Twose","sequence":"additional","affiliation":[{"name":"Instituto de Estudios de Mastocitosis de Castilla-La Mancha (CLMast)\u2014Spanish Reference Center for Mastocytosis, Hospital Virgen del Valle\u2014Complejo Hospitalario de Toledo, 45071 Toledo, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"In\u00e9s","family":"Torrado","sequence":"additional","affiliation":[{"name":"Instituto de Estudios de Mastocitosis de Castilla-La Mancha (CLMast)\u2014Spanish Reference Center for Mastocytosis, Hospital Virgen del Valle\u2014Complejo Hospitalario de Toledo, 45071 Toledo, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2775-3681","authenticated-orcid":false,"given":"Knut","family":"Brockow","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Allergy Biederstein, School of Medicine, Technical University of Munich, 80802 Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5389-9190","authenticated-orcid":false,"given":"Joanna","family":"Renke","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Hematology and Oncology, Medical University of Gda\u0144sk, 80-211 Gda\u0144sk, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ninela","family":"Irga-Jaworska","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Hematology and Oncology, Medical University of Gda\u0144sk, 80-211 Gda\u0144sk, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9504-9848","authenticated-orcid":false,"given":"Katarzyna","family":"Plata-Nazar","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Pediatric Gastroenterology, Allergology and Nutrition, Medical University of Gda\u0144sk, 80-211 Gda\u0144sk, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hanna","family":"\u0141ugowska-Umer","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Venereology and Allergology, Medical University of Gda\u0144sk, 80-211 Gda\u0144sk, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3613-1224","authenticated-orcid":false,"given":"Justyna","family":"Czarny","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Venereology and Allergology, Medical University of Gda\u0144sk, 80-211 Gda\u0144sk, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5791-0775","authenticated-orcid":false,"given":"Anna","family":"Belloni Fortina","sequence":"additional","affiliation":[{"name":"Pediatric Dermatology Unit, Department of Medicine DIMED, University of Padua, 35128 Padua, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3583-0816","authenticated-orcid":false,"given":"Francesca","family":"Caroppo","sequence":"additional","affiliation":[{"name":"Pediatric Dermatology Unit, Department of Medicine DIMED, University of Padua, 35128 Padua, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4768-1387","authenticated-orcid":false,"given":"Roman J.","family":"Nowicki","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Venereology and Allergology, Medical University of Gda\u0144sk, 80-211 Gda\u0144sk, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6286-0693","authenticated-orcid":false,"given":"Bogus\u0142aw","family":"Nedoszytko","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Venereology and Allergology, Medical University of Gda\u0144sk, 80-211 Gda\u0144sk, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marek","family":"Niedoszytko","sequence":"additional","affiliation":[{"name":"Department of Allergology, Medical University of Gda\u0144sk, 80-211 Gda\u0144sk, 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