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Seltene Akne-assoziierte Syndrome und deren Bedeutung für das Verständnis der Pathogenese der Akne

Uncommon acne-associated syndromes and their significance in understanding the pathogenesis of acne

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Zusammenfassung

Die Akne stellt ein interessantes Modell für die Untersuchung der Wechselwirkung zwischen Hormonen, angeborener Immunität, Entzündung und Wundheilung (Narbenbildung) dar. Die Erscheinungsformen und Beteiligung von Akne bei verschiedenen systemischen Erkrankungen und einigen seltenen Syndromen zeigt die Vielschichtigkeit von verschiedenen Akne-assoziierten Erkrankungen auf. Das „Synovitis, Akne, Pustulosis, Hyperostose, Osteitis“ (SAPHO)-Syndrom und das „Pyogene Arthritis, Pyoderma gangraenosum, Akne“ (PAPA)-Syndrom, werden beide als autoinflammatorische Erkrankungen angesehen und zeigen die entzündlichen Merkmale der Akne auf. Während das SAPHO-Syndrom genutzt werden kann, um die pathogene Rolle des Propionibacterium acnes als Kofaktor zu untersuchen, helfen das PAPA-Syndrom und das Apert-Syndrom, die genetische Prädisposition von Akne besser zu verstehen. Die genetischen Defekte der „Gain-of-function-Mutationen“ des FGFR2-Gens beim Apert-Syndrom und beim Aknenävus (Munro) erhärten die Hypothese, dass die Interaktion der „forkhead box Klasse O“ (FoxO)-vermittelten Regulation der Transkription mit der Androgenrezeptortransaktivierung sowie Insulin und Insulin-ähnlichem Wachstumsfaktor (IGF-1) entscheidend an der Pathogenese der Akne sind. Neue Biologika wie Tumornekrosefaktor (TNF)-α-Blocker und IL-1-Inhibitoren erscheinen vielversprechend, dem entzündlichen Prozess beim SAPHO- und PAPA-Syndrom entgegenwirken zu können. Es bleibt aber abzuwarten, ob auch Patienten mit ausschließlich schwerer Akne von diesen Therapieansätzen langfristig profitieren können.

Abstract

Acne is an intriguing model for the study of interactions between hormones, innate immunity, inflammation and wound healing (scarring). The manifestations and involvement of acne in different systemic diseases and some rare syndromes demonstrate its multifaceted nature. Synovitis-Acne-Pustulosis-Hyperostosis-Osteitis (SAPHO) and Pyogenic Arthritis-Pyoderma gangrenosum-Acne (PAPA) syndromes, both regarded as autoinflammatory diseases, highlight the attributes of inflammation in acne. While SAPHO syndrome can be used to explore the pathogenic role of Propionibacterium acnes in acne, PAPA syndrome and Apert syndrome can help understand the genetic influence on acne. The genetic defects in the gain-of-function of FGFR2 mutations in Apert syndrome and acne nevus of Munro lend further support to the hypothesis that the interaction of forkhead box class O (FoxOs)-mediated transcriptional regulation with androgen receptor transactivation and insulin/insulin like growth factor-1(IGF-1)-signaling is crucial in acne pathogenesis. Novel biologics, such as tumor necrosis factor (TNF) blockers and IL-1 inhibitors, appear promising in opposing the inflammation associated with SAPHO and PAPA syndromes, but it remains to seen if they can also improve severe acne particularly in the long term.

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Danksagung

Die Autoren danken dem Christine Kühne Center for Allergy Research and Education (CK-CARE) für die Unterstützung.

Interessenkonflikt

Der korrespondierende Autor gibt für sich und seine Koautoren an, dass kein Interessenkonflikt besteht.

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Authors and Affiliations

  1. Klinik für Dermatologie, National Taiwan University Hospital, Taipei, Taiwan, China

    J.-B. Hong

  2. Klinik und Poliklinik für Dermatologie und Allergologie, Technische Universität München, Biedersteinerstr. 29, 80802, München, Deutschland

    H. Prucha, M. Ziai, J. Ring &  W. Chen

  3. Christine Kühne Center for Allergy Research and Education (CK CARE), Technische Universität München, München, Deutschland

    H. Prucha & J. Ring

  4. Fachbereich Dermatologie, Umweltmedizin und Gesundheitstheorie, Universität Osnabrück, Osnabrück, Deutschland

    B. Melnik

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  1. J.-B. Hong
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  2. H. Prucha
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  3. B. Melnik
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  4. M. Ziai
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  5. J. Ring
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  6. W. Chen
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Corresponding author

Correspondence to W. Chen.

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___ ___

Der erstgenannte (J.-B. Hong) und der zweitgenannte Autor (H. Prucha) teilen sich die Erstautorenschaft.

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Hong, JB., Prucha, H., Melnik, B. et al. Seltene Akne-assoziierte Syndrome und deren Bedeutung für das Verständnis der Pathogenese der Akne. Hautarzt 64, 274–279 (2013). https://doi.org/10.1007/s00105-012-2460-6

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  • DOI: https://doi.org/10.1007/s00105-012-2460-6

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