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Neutrophilenreiche und pustulöse Dermatitiden

Neue autoinflammatorische Erkrankungen und Syndrome

Neutrophilic and pustular dermatoses

New autoinflammatory diseases and syndromes

  • Schwerpunkt: Entzündliche Dermatosen
  • Published:
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Zusammenfassung

Es werden nichtinfektiöse entzündliche Dermatosen mit überwiegend neutrophilen Infiltraten sowie pustulöse Dermatosen beschrieben, deren Infiltratdichte von spärlich (bei der Urtikaria) bis sehr dicht mit Leukozytoklasie (z. B. Sweet-Syndrom oder Pyoderma gangraenosum) variiert. Neutrophile Dermatosen werden durch diverse nichtinfektiöse Immunmechanismen ausgelöst. In den letzten Jahren wurden zudem vermehrt ursächliche Mutationen entdeckt (z. B. NLRC4-Mutation bei Cryopyrin-assoziierten periodischen Fiebersyndromen [CAPS] und anderen autoinflammatorischen Syndromen; IL36RN-Mutationen bei pustulöser Psoriasis) und entsprechende zielgerichtete Therapien entwickelt. Neutrophile Dermatosen sind von großer klinischer Bedeutung, da ihnen benigne oder maligne Erkrankungen zugrunde liegen können.

Abstract

This article reviews noninfectious inflammatory dermatoses with mainly neutrophilic infiltrates and the formation of pustules. The infiltrate containing neutrophils may either be mild as in urticaria or very dense, even with leukocytoclasia, as in Sweet syndrome or pyoderma gangrenosum. Neutrophilic infiltrates and pustular eruptions are caused by different noninfectious immunomechanisms. For some of them, mutations have been found (e.g. NLRC4 mutation in cryopyrin-associated periodic syndromes (CAPS) leading to activation of the inflammasome; IL36RN mutation in pustular psoriasis resulting in uncontrolled IL36 signaling). Neutrophilic dermatoses are of high interest, as they may be the cause of underlying benign or malignant conditions. In recent years, efficient targeted therapies have been developed.

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Authors and Affiliations

  1. Klinik und Poliklinik für Dermatologie und Allergologie am Biederstein, Technische Universität München, Biedersteiner Straße 29, 80802, München, Deutschland

    Sigrid M. C. Möckel

  2. Dermatohistologie, Hautklinik, Universitätsklinikum Münster, Münster, Deutschland

    Dieter Metze

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  1. Sigrid M. C. Möckel
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Correspondence to Sigrid M. C. Möckel.

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Interessenkonflikt

S. M. C. Möckel und D. Metze geben an, dass kein Interessenkonflikt besteht.

Für diesen Beitrag wurden von den Autoren keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.

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T. Mentzel, Friedrichshafen

E. Bierhoff, Bonn

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Möckel, S.M.C., Metze, D. Neutrophilenreiche und pustulöse Dermatitiden. Pathologe 41, 364–370 (2020). https://doi.org/10.1007/s00292-020-00784-x

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  • DOI: https://doi.org/10.1007/s00292-020-00784-x

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