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Mastozytosen im Kindesalter

Childhood-onset mastocytosis

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Zusammenfassung

Mastozytosen sind seltene Erkrankungen, die durch eine meist gutartige Vermehrung und Akkumulation von Mastzellen in verschiedenen Geweben charakterisiert sind. Kinder sind vornehmlich von einer isolierten Hautmastozytose betroffen, die sich in einigen Aspekten von der kutanen Mastozytose des Erwachsenenalters unterscheidet. Mit Ausnahme der seltenen diffusen kutanen Mastozytose des Kindesalters lassen sich in der Regel bei der kindlichen Mastozytose keine Mastzellinfiltrate im Knochenmark oder anderen extrakutanen Organsystemen nachweisen. Die Prognose der kindlichen Mastozytose ist im Regelfall sehr günstig. Im Gegensatz zu Mastozytosen des Erwachsenenalters, die meist chronisch-stabil oder langsam progredient verlaufen, sind die Mastozytosen des Kindesalters häufig transient und selbstlimitierend. In dieser Übersicht werden die verschiedenen Formen kindlicher Mastozytosen, aktuelle Erkenntnisse zu deren Pathogenese sowie Gemeinsamkeiten und Unterschiede zu Mastozytosen des Erwachsenenalters dargestellt.

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.

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Danksagung

Wir danken Nicole Schoepke, Tatevik Ohanyan, Johanna Meinhard und Karola Stieler für die kritische Durchsicht und Korrektur des Manuskripts.

Interessenkonflikt

Der korrespondierende Autor gibt an, dass kein Interessenkonflikt besteht.

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Authors and Affiliations

  1. Interdisciplinary Mastocytosis Center Charité, Klinik für Dermatologie, Venerologie und Allergologie, Charité – Universitätsmedizin Berlin, Charitéplatz 1, 10117, Berlin, Deutschland

    F. Siebenhaar, K. Weller, U. Blume-Peytavi & M. Maurer

Authors
  1. F. Siebenhaar
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  2. K. Weller
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  3. U. Blume-Peytavi
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  4. M. Maurer
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Correspondence to F. Siebenhaar.

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Siebenhaar, F., Weller, K., Blume-Peytavi, U. et al. Mastozytosen im Kindesalter. Hautarzt 63, 104–111 (2012). https://doi.org/10.1007/s00105-011-2201-2

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  • DOI: https://doi.org/10.1007/s00105-011-2201-2

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