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Pyoderma gangraenosum und Sweet-Syndrom

Hautmanifestationen autoinflammatorischer Erkrankungen

Pyoderma gangrenosum and Sweet’s syndrome

Cutaneous manifestations of autoinflammatory disorders

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Zusammenfassung

Das Pyoderma gangraenosum und das Sweet-Syndrom sind seltene Erkrankungen aus der Gruppe der neutrophilen Dermatosen und teilen eine Vielzahl an Charakteristika. Zwar unterscheiden sich die beiden Erkrankungen klinisch deutlich voneinander, jedoch zeigen beide in der Histologie ausgeprägte dermale Infiltrate mit neutrophilen Granulozyten ohne Hinweis auf eine primäre Vaskulitis und sprechen gut auf immunsuppressive Medikamente an. Zudem sind beide Krankheiten häufig assoziiert mit anderen systemischen und hämatologischen Erkrankungen. Neueste Erkenntnisse zeigen, dass die neutrophilen Dermatosen als kutane Manifestation von Autoinflammation betrachtet werden können, was einen interessanten neuen Aspekt bei der Entstehung der beiden Erkrankungen und neue Therapieansätze aufzeigt.

Abstract

Pyoderma gangrenosum and Sweet’s syndrome are rare diseases that belong to the group of neutrophilic dermatoses and share several common characteristics. Although the two disorders differ clinically from each other, both diseases show pronounced dermal infiltration of neutrophils without evidence of primary vasculitis and respond well to immunosuppressive drugs. In addition, both diseases are often associated with other systemic and hematological disorders. Recent findings show that the neutrophil dermatoses can be considered as cutaneous manifestations of autoinflammation, demonstrating an interesting new aspect in the development of the diseases and additional therapeutic avenues.

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Authors and Affiliations

  1. Klinik für Dermatologie, Universitätsspital Zürich, Gloriastr. 31, 8091, Zürich, Schweiz

    B. Meier,  J.-T. Maul &  L. E. French

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  1. B. Meier
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  2. J.-T. Maul
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  3. L. E. French
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Correspondence to B. Meier.

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Interessenkonflikt

B. Meier, J.-T. Maul und L.E. French geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

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Meier, B., Maul, JT. & French, L.E. Pyoderma gangraenosum und Sweet-Syndrom. Hautarzt 67, 934–939 (2016). https://doi.org/10.1007/s00105-016-3888-x

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  • DOI: https://doi.org/10.1007/s00105-016-3888-x

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