Hidradenitis suppurativa

Zuletzt aktualisiert: 2024-09-29

Autor(en): Anzengruber F., Navarini A.

ICD11: ED92.0

1/13

  • Follikulitis profunda (Furunkel/Karbunkel)
  • Prurigo simplex subacuta Hebra
  • Bakterielle Pyodermie
  • Pyoderma gangraenosum
  • Pyoderma vegetans
  • Ecthyma
  • Akne vulgaris (comedonica, papulopustulosa, conglobata)
  • Plattenepithelzellkarzinom der Haut (Spinozelluläres Karzinom, SCC)

Verneuil 1854, Plewig & Steger 1989 

Akne inversa, Verneuil's disease

Chronische-rezidiverende Entzündung der Haarfollikel (Terminalhaare) in den intertriginösen Areale mit Komedo-, Abszess-, Fistel- und Narbenstrangbildung. Es handelt sich nicht um eine primäre Schweissdrüsen-Entzündung.

  • Prävalenz: 1%
  • Inzidenz: Unterschiedliche Daten, whs. 1:600
  • Häufigkeitsgipfel: 28- 64 Jahre
  • Frauen > Männer (kontrovers diskutiert)

Hurley Grad Abszesse Narbenstränge Flächiger Befall
I + - -
II + + -
III + + +
  • Akne-Triade
    • Acne conglobata
    • Hidradenitis suppurativa
    • Perifolliculitis capitis abscedens et suffodiens
  • Akne-Tetrade
    • Acne conglobata
    • Hidradenitis suppurativa
    • Perifolliculitis capitis abscedens et suffodiens
    • Pilonidalsinus

  • Sowohl keratinozytäre Hyperproliferation in den Infundibula als auch autoinflammatorische Aspekte
  • Assoziationen
    • Psoriasis
    • Chronisch entzündliche Darmerkrankungen
    • Rheumafaktor-negative Polyarthritiden
    • Hypertonie
    • Pyoderma gangraenosum
    • Systemische Amyloidose
  • Prädispositionsfaktoren:
    • Nikotinkonsum
    • Adipositas
    • Enge Kleidung bzw. Reibung
    • ev. Hyperhidrose (kontrovers diskutiert)

  • Auftreten von inflammatorischen Knoten und Abszessen im Bereich der Intertrigines, genital und selten um die Brustwarzen
  • Die follikuläre Hyperkeratose (nicht immer vorhanden) führt zur Bildung von Komedonen

  • Eindeutige Klinik (mind. 2 Abszesse innerhalb von 6 Monaten im Bereich der Intertrigines)
  • Vorhandensein von Risikofaktoren kombiniert mit der Klinik
  • Restliche Akne-Tetrade muss nicht vorhanden sein, unterstützt aber die Diagnose

 

Hurley Score: 

  1. Einzelne oder multiple Abszesse ohne Vernarbung oder Fisteln
  2. Rezidivierende Abszesse, einzeln oder weit auseinanderstehende, mit Fistelbildung
  3. Diffuses oder breitbasiges Auftreten, multiple verbundene Läsionen mit Fisteln und Abszessen. Narben können zur Bewegungseinschränkung führen, sowie zur funktionellen Beeinträchtigung (Genito-Analbereich).

 

IHS4-Score ausrechnen: Anzahl der Knoten x1, Anzahl der Abszesse x2, Fisteln / Sinus x4

<4 Punkte: milde HS, 4-10 Punkte: mittelstarke HS, >10 Punkte: starke HS

Axillär, Inguinal, Perianal, Perineum, Skrotum, Submammär, Retroaurikulär.

  • Rezidiv post OP
    • Axillär ca. 3%
    • Inguino-perineal ca. 35%
    • Submammär ca. 50%
  • Pararektale Fisteln
  • Urethrale Fisteln
  • Bewegungseinschränkungen
  • Genitales Lymphödem
  • Plattenepithelkarzinome (Marjolin-Ulcus)
  • Höheres Risiko von cerebrovaskulärem Insult, koronare Herzkrankheit, Herzversagen, PAVK

  • Gewichtsreduktion
  • Lockere, nicht reibende Kleider
  • Vermeidung Nikotinabusus

Chronisch, rezidivierende Krankheit. Durch Reduktion von Uebergewicht und Sistierung des Nikotinabusus kann es bei manchen Patienten eine deutliche Besserung und Abheilung geben (schon mehrfach beobachtet). Dies ist hingegen kaum zu erwarten bei denjenigen Formen, die ohne vorherige Präsenz der Risikofaktoren auftreten.

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