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Aktuelle Dermatologie 2015; 41(05): 185-199
DOI: 10.1055/s-0034-1391994
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© Georg Thieme Verlag KG Stuttgart · New York

Hidradenitis suppurativa / Acne inversa[*]

Aktuelles zur Definition, Epidemiologie, Pathogenese, Klassifikation und Evidenz-basierten TherapieHidradenitis suppurativa / Acne inversaCurrent Aspects on Definition, Epidemiology, Pathogenesis, Classification and Evidence-based Treatment
C. C. Zouboulis
1   Klinik für Dermatologie, Venerologie und Allergologie / Immunologisches Zentrum, Städtisches Klinikum Dessau
2   European Hidradenitis Suppurativa Foundation e. V., Dessau, Europe
,
M. Brunner
1   Klinik für Dermatologie, Venerologie und Allergologie / Immunologisches Zentrum, Städtisches Klinikum Dessau
,
U. Lippert
1   Klinik für Dermatologie, Venerologie und Allergologie / Immunologisches Zentrum, Städtisches Klinikum Dessau
,
P. Seele
1   Klinik für Dermatologie, Venerologie und Allergologie / Immunologisches Zentrum, Städtisches Klinikum Dessau
,
D. Trebing
1   Klinik für Dermatologie, Venerologie und Allergologie / Immunologisches Zentrum, Städtisches Klinikum Dessau
› Institutsangaben
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Publikationsverlauf

Publikationsdatum:
21. Mai 2015 (online)

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Zusammenfassung

Hidradenitis suppurativa / Acne inversa (HS) ist eine chronisch rezidivierende Hauterkrankung (des terminalen Haarfollikels), die üblicherweise nach der Pubertät auftritt und vernarbend verlaufen kann. Sie manifestiert sich mit schmerzhaften, tief lokalisierten, entzündlichen Hautläsionen, die in apokrinen Drüsen-reichen Hautregionen auftreten, am häufigsten in den Axillen sowie der Inguinal- und Anogenitalregion (Dessauer Definition). Die berichtete Prävalenz beträgt 1 % in Europa und 0,05 % in den USA, die Inzidenz in den USA liegt bei 6 Patienten pro 100 000 Einwohner und Jahr mit steigender Tendenz. Frauen sind häufiger von HS befallen. Es besteht eine starke Assoziation der Erkrankung mit aktivem Rauchen (relatives Risiko [RR] = 12,6) und erhöhtem Körpermassenindex (RR = 1,1 für jede BMI-Einheit). Die primären diagnostischen Kriterien sind rezidivierende schmerzhafte oder eitrige Läsionen mehr als 2-mal in 6 Monaten in der Anamnese und die Beteiligung von Achselhöhlen, Leisten, Damm, Gesäßbereich und dem submammären Bereich bei der Frau. Es treten Knötchen, Abszesse, Fistelgänge und Narbenbildung auf. Es gibt keinen biologischen diagnostischen Marker. Die primären Veränderungen betreffen nicht, wie ursprünglich vermutet, die apokrine Drüse, sondern den Haarfollikel. Das zentrale pathogenetische Ereignis stellt eine Hyperkeratose mit Verschluss des Haarfollikels dar. Entzündliche Darmerkrankungen und Spondylarthropathien treten häufiger zusammen mit einer HS auf. Auch das metabolische Syndrom wurde in Verbindung mit HS gebracht. Die HS zeigt einen variablen klinischen Verlauf, der in 3 Schweregrade nach Hurley klassifiziert wird. Die HS verursacht eine deutliche negative Beeinflussung der Lebensqualität der Patienten, sie stellt sogar die am stärksten die Lebensqualität belastende dermatologische Erkrankung dar. Das Ziel der Behandlung ist die Reduktion der aktiven Entzündung und der Krankheitsrezidive bis zum vollständigen Sistieren der Beschwerden. Die chirurgische Behandlung bleibt die tragende Säule bei der Behandlung sowohl von einzelnen, tiefen und vernarbenden Läsionen als auch ausgedehnten HS-Formen. Dabei verhalten sich Rezidivrisiko und Radikalität des Eingriffs zueinander umgekehrt proportional. Das Ziel der konservativen Therapie ist eine Optimierung des präoperativen Hautzustandes. Zurzeit gibt es kein zugelassenes Medikament für die konservative HS-Therapie. Die höchste therapeutische Evidenz weisen die chirurgische Exzision, Adalimumab s. c., Clindamycin-Rifampicin p. o., Infliximab i. v., hormonelle Antiandrogene (bei der Frau), der vaporisierende Nd:YAG-Laser (Hurley-Grad II), Clindamycin-Lösung/Gel lokal (Hurley-Grad I) und der Gentamycin-Schwamm (postoperativ, lokal) auf.

Abstract

Hidradenitis suppurativa / acne inversa (HS) is a chronic recurrent skin disease (of the terminal hair follicle), which usually occurs after puberty with a potential scarring course. It presents with painful, deeply localized inflammatory skin lesions that occur in apocrine gland-rich regions of the skin, most commonly in the axillary, inguinal and anogenital regions (Dessauer definition). The reported prevalence is 1 % in Europe and 0.05 % in the USA; the incidence in the USA is 6 patients/100,000 inhabitants per year with an increasing trend. Women are mostly affected. There is a strong association of the disease with active smoking (relative risk [RR] = 12.6) and increased body mass index (RR = 1.1 for each BMI unit). The primary diagnostic criteria include recurrent painful or purulent lesions more than 2 ×/6 months in the history and the involvement of armpits, groins, perineum, buttocks area and submammary region in women. Characteristic clinical signs are nodules, abscesses, sinus tracts, and scarring. There is no biological diagnostic marker. The primary changes do not affect, as originally suspected, the apocrine gland but the hair follicle. The central pathogenetic event corresponds to a hyperkeratosis with occlusion of the hair follicle. Inflammatory bowel disease and spondyloarthropathies occur together with HS quite frequently. The metabolic syndrome has been associated with HS. The HS presents a variable clinical course, which is classified into three severity grades according to Hurley. HS causes a strong negative effect on the quality of patients' life, it even constitutes the most substantial burden of quality of life of the dermatological diseases. The goal of treatment is to reduce active inflammation and decrease disease recurrences until the complete cessation of symptoms. Surgical treatment remains the mainstay in the treatment of both, individual, deep and scarring lesions as well as for the extended HS forms. Recurrence rates and radicality of surgery are inversely proportional to each other. The goal of conservative therapy is the optimization of preoperative skin condition. Currently there are no approved drugs for conservative HS therapy in Germany. The highest therapeutic evidence is shown for surgical excision, Adalimumab s. c., clindamycin/rifampicin p. o., infliximab i. v., hormonal antiandrogens (in females), vaporizing Nd:YAG Laser (Hurley II), clindamycin solution/gel locally (Hurley I) and gentamicin sponges (postoperatively, locally).

* Herrn Prof. Dr. Hans-Dieter Göring zum 75. Geburtstag gewidmet


 

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