Hidradenitis suppurativa

Zuletzt aktualisiert: 2024-12-03

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

ICD11: ED92.0

1/13

Verneuil 1854, Plewig & Steger 1989 

Akne inversa, Verneuil's disease

Chronisch-rezidivierende potentiell mutilierende Hauterkrankung des terminalen Haartalgdrüsenapparats in den apokrinen drüsenreichen Körperregionen vor allem in den intertriginösen Arealen, die sich mit schmerzhaften, entzündlichen Läsionen manifestiert. 

  • Prävalenz: ca. 0.4%-1% weltweit
  • Inzidenz : 6.0 pro 100 000 Einwohner
  • selten vor der Pubertät oder nach der Menopause, das durchschnittliche Alter bei Erstmanifestation liegt bei 22–23 Jahren
  • häufiger bei dunkelhäutigen Menschen und Menschen mit niedrigem sozioökonomischem Status
  • Frauen-Männer Ratio 1:2 bis 1:5 (kontrovers diskutiert)

Aktive, entzündliche Form: IHS4- Klassifikation

 

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

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

 

Inaktive, Nicht entzündliche Form

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. 

 

 Hurley Grad   Abszesse   Narbenstränge   Flächiger Befall 
I + - -
II + + -
III + + +

 

Die zwei Säulen der HS/AI-Pathogenese: 

  1. eine abnormale Differenzierung der Keratinozyten des Haartalgdrüsenapparats 
  2. eine massive begleitende Entzündung.

 

eine abnormale Differenzierung der Keratinozyten des Haartalgdrüsenapparats -> Hyperkeratose mit Verschluss des oberen Anteils des Haarfollikels -> Dilatation -> Ruptur des Follikels -> eitrig-verschmelzender Entzündungsreaktion -> Vernarbung -> Sinusformation

 

  • Prädispositionsfaktoren:
    • Nikotinkonsum
    • Adipositas
    • Enge Kleidung bzw. Reibung
    • Hyperhidrose
    • die genetische Prädisposition (34% der Verwandten ersten Grades von HS/AI-Patienten ebenfalls an HS/AI leiden)

 

  • Komorbidität:
    • Psoriasis
    • Chronisch entzündliche Darmerkrankungen
    • Axiale Spondyloarthritis
    • Kardiovaskuläre Erkrankungen
    • Pyoderma gangraenosum
    • Diabetes mellitus
    • Psychiatrische Erkrankungen
    • Akne-Triade (Acne conglobata,HS, Perifolliculitis capitis abscedens et suffodiens)
    • Akne-Tetrade (Acne conglobata, HS, Perifolliculitis capitis abscedens et suffodiens, Pilonidalsinus (23-30%))

 

Syndromale HS/AI:

  • im Rahmen komplexer (autoinflammatorischer) Syndrome wie u.a. PASH, PAPASH, PASS und PsAPASH
  • stärker ausgeprägt und therapierefraktärer
  • individuelle Empfehlung zur Behandlung (genetische Anomalien in für IL-1- und TNF-α-Signalwege)

 

  • Die primäre Läsion: ein schmerzhafter, solitär tief sitzender, kutan-subkutaner Knoten, der sich spontan zurückbildet, persistiert oder sich in einen Abszess umwandeln kann.
  • Abszesse können in der Tiefe verschmelzen, nach aussen spontan rupturieren und Tunnel bilden. Die Abszess- bzw. Tunnelruptur geht mit einer eitrigen und/oder wässrig-blutigen Sekretion einher.
  • Weitere Präsentationen: follikuläre Papeln, Zysten, Pusteln, schmale, intraepidermale Tunnel, die doppelte Pseudokomedonen, grossflächigen, teils livid-erythematösen, knotigen, honigwabenartigen Arealen mit Abszessen, Tunnelgängen und hypertrophe Narben
  • Symptomatische Beschwerden: Schmerzen, Juckreiz, übel-riechender Ausfluss.

Eindeutige Klinik mit mind. 2 Abszesse innerhalb von 6 Monaten im Bereich der Intertrigines

Inguinal (90%), axillär (69%), perianal und perineal (37%), gluteal (27 %), submammär (18 %), genitofemoral, im Mons pubis und seltener im Gesicht, thorakal, retroaurikulär, am Kapillitium, den Augenlidern und am Rücken

Die Hidradenitis suppurativa / Acne inversa wurde 1839 von Velpeau beschrieben und 1854 von Verneuil benannt, der sie mit Schweissdrüsen assoziierte. 1989 führten Plewig und Steger den Begriff Acne inversa ein, um den irreführenden Begriff Hidradenitis suppurativa zu ersetzen. Beide Bezeichnungen gelten aktuell als inkorrekt und werden parallel verwendet, bis ein passenderer Name aufgrund eines besseren Verständnisses der Pathogenese gefunden wird.

Das entzündliche Infiltrat ist vielfältig und besteht aus Lymphozyten, Histiozyten, Fremdkörperriesenzellen, Plasmazellen sowie neutrophilen Granulozyten. Abhängig vom Entzündungsgrad und Stadium der HS/AI-Läsionen können unterschiedliche histopathologische Befunde festgestellt werden. Das histologische Bild ist jedoch meist eindeutig, sodass klinisch schwer abzugrenzende Differenzialdiagnosen mit relativer Sicherheit ausgeschlossen werden können.

  • Chronische Schmerzen
  • Kutane Superinfektionen
  • Pararektale Fisteln
  • Urethrale Fisteln
  • Bewegungseinschränkungen
  • Lymphödem, vor allem genital
  • Plattenepithelkarzinome (Marjolin-Ulcus) (frühes, hohes Metastasierungsrisiko, schlechte Prognose (59% Mortalität)
  • Höheres Risiko von cerebrovaskulärem Insult, koronare Herzkrankheit, Herzversagen, PAVK
  • systemische Amyloidose mit anschliessender Nieren-, Herz- und Zentralnervensystemschädigung (Screening: Microalbuminurie-> ggf. Nierenbiopsie)
  • Anämie
  • Hypoproteinämie

  • Gewichtsreduktion
  • Lockere, nicht reibende Kleider
  • Vermeidung Nikotinabusus
  • Langgepulster Nd:YAG-Laser (zu Haarentfernung)

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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