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Pathogenese der Hidradenitis suppurativa/Acne inversa

Pathogenesis of hidradenitis suppurativa/acne inversa

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Zusammenfassung

Die Hidradenitis suppurativa (HS)/Acne inversa (AI) weist eine multifaktorielle Pathogenese auf. Neben einer sporadischen Form wird bei ca. 40 % der Patienten eine familiäre Form berichtet, für die ein autosomal-dominanter (AD) Erbgang mit einer reduzierten Genpenetranz angenommen wird. Der Phänotyp der Erkrankung mit entzündlichen Knoten, Abszessen und im Verlauf sezernierenden Fisteln lässt eine infektiöse Genese vermuten, allerdings bleibt bis heute die genaue Rolle der nachgewiesenen Bakterien bei der Pathogenese der HS unklar. Rauchen und das metabolische Syndrom werden als wichtige Triggerfaktoren der HS angesehen, wobei beim Letzteren hauptsächlich Adipositas und hormonelle Veränderungen eine pathogenetische Rolle spielen. Letztlich sind bei der überschießenden Entzündungsreaktion der HS Toll-like-Rezeptoren, antimikrobielle Peptide, Immunzellen und Schlüsselzytokine beteiligt, die auch die Ziele zukünftiger Therapien darstellen.

Abstract

Hidradenitis suppurativa/acne inversa (HS) has a multifactorial pathogenesis. In addition to a sporadic form, a familial form is reported in around 40% of patients, for whom an autosomal dominant (AD) inheritance with reduced gene penetrance is assumed. The phenotype of the disease with inflammatory nodules, abscesses and secreting sinus tracts suggests an infectious origin, but the exact role of the bacteria detected in HS pathogenesis remains unclear. Smoking and metabolic syndrome are regarded as important trigger factors in HS, with obesity and hormonal changes playing a pathogenic role in the latter. Ultimately, Toll-like receptors, antimicrobial peptides, immune cells and key cytokines are involved in the excessive inflammatory reaction of HS and are also the targets of future therapies.

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Authors and Affiliations

  1. Hochschulklinik für Dermatologie, Venerologie und Allergologie, Immunologisches Zentrum, Städtisches Klinikum Dessau, Medizinische Hochschule Brandenburg Theodor Fontane und Fakultät für Gesundheitswissenschaften Brandenburg, Auenweg 38, 06847, Dessau, Deutschland

    G. Nikolakis & C. C. Zouboulis

  2. European Hidradenitis Suppurativa Foundation e. V., Dessau, Deutschland

    G. Nikolakis, G. Kokolakis, K. Wolk, R. Hunger, R. Sabat & C. C. Zouboulis

  3. Psoriasis Forschungs- und BehandlungsCentrum, Klinik für Dermatologie, Venerologie und Allergologie und Institut für Medizinische Immunologie, Charité – Universitätsmedizin Berlin, Berlin, Deutschland

    G. Kokolakis, K. Wolk & R. Sabat

  4. Interdisziplinäre Gruppe Molekulare Immunpathologie, Dermatologie/Med. Immunologie, Charité – Universitätsmedizin Berlin, Berlin, Deutschland

    G. Kokolakis, K. Wolk & R. Sabat

  5. Klinik für Dermatologie und Venerologie, Jagiellonen-Universität, Krakow, Polen

    K. Kaleta

  6. Klinik für Dermatologie, Inselspital, Bern Universitätsklinikum, Bern Universität, Bern, Schweiz

    R. Hunger

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  1. G. Nikolakis
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G. Nikolakis, G. Kokolakis, K. Kaleta und R. Hunger geben an, dass kein Interessenkonflikt besteht. K. Wolk erhielt Projektunterstützung, Auszeichnungen oder Vergütung für Tätigkeiten im Rahmen klinischer Studien, Referenten- oder Beratertätigkeiten von folgenden Firmen (in alphabetischer Reihenfolge): AbbVie, Biogen IDEC, Bristol Myers Squibb, Celgene, Charité Research Organisation, Dr. Willmar Schwabe, Flexopharm, Janssen-Cilag, Novartis Pharma, Pfizer, Sanofi-Aventis, TFS Trial Form Support und UCB Pharma. R. Sabat erhielt Projektunterstützung, Auszeichnungen oder Vergütung für Tätigkeiten im Rahmen klinischer Studien, Referenten- oder Beratertätigkeiten von folgenden Firmen (in alphabetischer Reihenfolge): AbbVie, AMGEN, Bayer, Biogen IDEC, Boehringer Ingelheim Pharma, Bristol Myers Squibb, Celgene, Charité Research Organisation, CSL Behring, Dr. Willmar Schwabe, Flexopharm, Janssen-Cilag, La Roche-Posay Laboratoire Dermatologique, Novartis Pharma, Parexel, Pfizer, Sanofi-Aventis, TFS Trial Form Support und UCB Pharma. C.C. Zouboulis hat Berater- und Vortragshonorare von Idorsia, Incyte, InflaRx, Janssen, Novartis, Regeneron und UCB erhalten, die nicht mit diesem Manuskript in Verbindung stehen. Seine Abteilung erhielt Honorare von AbbVie, InflaRx, Novartis und UCB für seine Teilnahme an klinischen Studien.

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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Nikolakis, G., Kokolakis, G., Kaleta, K. et al. Pathogenese der Hidradenitis suppurativa/Acne inversa. Hautarzt 72, 658–665 (2021). https://doi.org/10.1007/s00105-021-04853-x

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  • DOI: https://doi.org/10.1007/s00105-021-04853-x

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