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Mastocytosis

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Pruritus

Abstract

Mastocytosis is a heterogeneous disease characterized by tissue accumulation of clonal mast cells. Variants range from cutaneous mastocytosis and indolent systemic mastocytosis with normal life expectancy to rare aggressive forms with a fatal outcome. The WHO classification of disease variants and common clinical symptoms such as itching, flushing, diarrhea and hypotension caused by mast cell mediator release are presented, and the role of mast cell activation and its triggers are described. Differences in disease presentation between adults and children are highlighted. Typical diagnostic approaches involving skin and bone marrow biopsies and blood and urine analyses of mast cell mediator levels are provided and a selection of therapy regimes extending beyond antihistamines as the basis of anti-mediator therapy are summarized.

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Abbreviations

ASM:

Aggressive SM

CM:

Cutaneous mastocytosis

H-receptor:

Histamine receptor

ISM:

Indolent systemic mastocytosis

MCL:

Mast cell leukemia

MIS:

Mastocytosis in the skin

NT:

Neurotrophin

SCF:

Stem cell factor

SM:

Systemic mastocytosis

SM-AHNDM:

SM with associated clonal hematological non-mast cell lineage

TMEP:

Telangiectasia macularis eruptive perstans

WHO:

World Health Organization

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Authors and Affiliations

  1. Department of Dermatology, Venereology and Allergology, University Medical Center, Georg August University, Robert-Koch-Str. 40, Goettingen, Lower Saxony, 37075, Germany

    Undine Lippert MD, PhD

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  1. Undine Lippert MD, PhD
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Correspondence to Undine Lippert MD, PhD .

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Editors and Affiliations

  1. Department of Dermatology, University of Brest, Brest, France

    Laurent Misery

  2. Center of Chronic Pruritus, University Hospital Münster, Münster, Nordrhein-Westfalen, Germany

    Sonja Ständer

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Lippert, U. (2016). Mastocytosis. In: Misery, L., Ständer, S. (eds) Pruritus. Springer, Cham. https://doi.org/10.1007/978-3-319-33142-3_22

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  • DOI: https://doi.org/10.1007/978-3-319-33142-3_22

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