title: [Childhood-onset mastocytosis].
abstract: Mastocytoses are a group of rare diseases characterized, in most cases, by a benign proliferation and accumulation of mast cells in different tissues. In children, mastocytosis affects usually exclusively the skin and differs in many aspects from adult-onset mastocytosis. Except for diffuse cutaneous mastocytosis, which is an uncommon subtype of childhood-onset mastocytosis, involvement of the bone marrow or additional extracutaneous organs is rarely seen in children. The prognosis of childhood-onset mastocytosis is usually very good. Compared to adult patients who most commonly experience chronic-stable or slowly progressive disease, mastocytosis in children is mostly transient and self-limiting. In this review, we present and discuss the subtypes of childhood-onset mastocytosis, recent advances in the understanding of their pathogenesis as well as similarities and differences between adult- and childhood-onset mastocytosis.
authors.affiliation: Interdisciplinary Mastocytosis Center Charité, Klinik für Dermatologie, Venerologie und Allergologie, Charité – Universitätsmedizin Berlin, Charitéplatz 1, 10117 Berlin. frank.siebenhaar@charite.de
authors.forename: F
authors.initials: F
authors.lastname: Siebenhaar
authors.forename: K
authors.initials: K
authors.lastname: Weller
authors.forename: U
authors.initials: U
authors.lastname: Blume-Peytavi
authors.forename: M
authors.initials: M
authors.lastname: Maurer
mesh_terms: D002648:Child; D002675:Child, Preschool; D003879:Dermatologic Agents / Q000627:therapeutic use*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D034701:Mastocytosis, Cutaneous / Q000175:diagnosis* / Q000628:therapy*
chemical_list: D003879:Dermatologic Agents