Abstract
Mastocytosis is characterized by clonal expansion of mast cells. When diagnosed in children, mastocytosis is usually limited to the skin, which is termed cutaneous mastocytosis (CM). According to its clinical presentation, CM can be classified into mastocytoma (20% of cases), maculopapular CM (MPCM, 75%; previously urticaria pigmentosa), and diffuse cutaneous mastocytosis (DCM; 5%). 1 Hartmann K. Escribano L. Grattan C. et al. Cutaneous manifestations in patients with mastocytosis: consensus report of the European Competence Network on Mastocytosis; the American Academy of Allergology, Asthma & Immunology; and the European Academy of Allergology and Clinical Immunology. J Allergy Clin Immunol. 2016; 137: 35-45 Abstract Full Text Full Text PDF PubMed Scopus (176) Google Scholar ,2 Méni C. Bruneau J. Georgin-Lavialle S. et al. Paediatric mastocytosis: a systematic review of 1747 cases. Br J Dermatol. 2015; 172: 642-651 Crossref PubMed Scopus (104) Google Scholar Contrary to adult-onset mastocytosis, complete or partial resolution is often noted by adolescence. 3 Carter M.C. Clayton S.T. Komarow H.D. et al. Assessment of clinical findings, tryptase levels, and bone marrow histopathology in the management of pediatric mastocytosis. J Allergy Clin Immunol. 2015; 136: 1673-1679 Abstract Full Text Full Text PDF PubMed Scopus (52) Google Scholar Serum tryptase level is a marker of mast cell burden. It is widely available and used to assess risk of systemic involvement. Other causes of elevated basal tryptase levels include chronic eosinophilic leukemia, renal disease, and hereditary α-tryptasemia. 4 Butterfield J.H. Ravi A. Pondee T. Mast cell mediators and significance in clinical practice in mastocytosis. Immunol Allergy Clin N Am. 2018; 38: 397-410 Abstract Full Text Full Text PDF PubMed Scopus (17) Google Scholar
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