S2k-Leitlinie zur Therapie der Hidradenitis suppurativa/Acne inversa (ICD-10-Code: L73.2)

Zusammenfassung

Ziel der S2k-Leitlinie zur Therapie der Hidradenitis suppurativa/Acne inversa (HS/AI) ist es, eine akzeptierte Entscheidungshilfe für die Auswahl sowie Durchführung einer geeigneten/suffizienten Therapie von Patienten mit HS/AI zu liefern. HS/AI ist eine chronisch rezidivierende, entzündliche, potenziell mutilierende Hauterkrankung des terminalen Haartalgdrüsenapparats, die sich mit schmerzhaften, entzündlichen Läsionen in den apokrinen drüsenreichen Körperregionen manifestiert, insbesondere in der axillären sowie der Inguinal- und Anogenitalregion. Die intensive klinische und experimentelle Forschung sowie Überprüfung neuer therapeutischen Ansätze haben seit der Veröffentlichung der alten Leitlinie im Jahr 2012 die Kenntnisse auf dem Gebiet der HS/AI bedeutend verbessert. In Deutschland wurde eine Punktprävalenz der aktiven Erkrankung von 0,3 % beobachtet, sie stieg unter Berücksichtigung von Patienten mit HS/AI-typischen Narben aber nicht aktiver Erkrankung auf 3,0 %. Säulen der HS/AI-Pathogenese sind eine abnormale Differenzierung der Keratinozyten des Haartalgdrüsenapparats und eine massive begleitende Entzündung. Die primären Läsionen der HS/AI sind entzündliche Knoten, Abszesse und drainierende Tunnel, überwiegend an den Prädilektionsstellen (axillär, submammär, inguinal, genital und perineal). Rezidive in den letzten 6 Monaten mit mindestens 2 Läsionen an den Prädilektionsstellen verweisen auf eine HS/AI. Obwohl anhand dieser Kriterien eine klinische Diagnosestellung mit einer hohen Genauigkeit von 97 % erfolgen kann, ist die Erkrankung noch wenig bekannt, wie die Verspätung der Diagnose in Deutschland von 10,0 ± 9,6 Jahren beweist. Patienten mit HS/AI leiden an einer deutlichen Einschränkung der Lebensqualität, insbesondere bei hohem Morbiditätsgrad. HS/AI soll in der täglichen Praxis mit einem validierten Instrument klassifiziert und ihre Aktivität bewertet werden, v. a. mit dem International Hidradenitis Suppurativa Severity Scoring System (IHS4), um korrekte Therapieentscheidungen treffen zu können. Die HS/AI wird in 2 Formen im Zusammenhang mit der Ausprägung der stets nachweisbaren Entzündung eingeteilt, nämlich der entzündlichen und der vorwiegend nicht entzündlichen Formen. Während die Intensität der entzündlichen Form mithilfe der IHS4-Klassifikation in milde, mittelschwere und schwere HS/AI eingeteilt und entsprechend medikamentös behandelt wird, wird für die vorwiegend nicht entzündliche Form über eine chirurgische Behandlung nach dem Hurley-Grad der befallenen Lokalisation, nämlich Hurley-Grad I, II und III entschieden. Orale Tetrazykline oder eine 5-tägige intravenöse Therapie mit Clindamycin sind der Effektivität der oralen systemischen Kombination von Clindamycin und Rifampicin gleich gestellt. Die subkutan applizierbaren monoklonalen Antikörper Adalimumab und Secukinumab sind für die Therapie der HS/AI zugelassen. Für die vorwiegend nicht entzündliche Form der Erkrankung stehen verschiedene operative Verfahren zur Verfügung. Die Kombination einer medikamentösen Therapie zur Reduktion der Entzündung mit einem operativen Verfahren, zur Beseitigung des irreversiblen Gewebeschadens, gilt aktuell als ganzheitliches Therapieverfahren bei HS/AI. Eine regelmäßige Kontrolle und ggf. Anpassung der Therapie im Hinblick auf einen sich ändernden Krankheitsschweregrad wird empfohlen.

Abstract

The aim of the S2k guideline on the therapy of hidradenitis suppurativa/acne inversa (HS/AI) is to provide an accepted decision-making aid for the selection and implementation of a suitable/sufficient therapy for patients with HS/AI. HS/AI is a chronic, relapsing, inflammatory, potentially mutilating skin disease of the terminal pilosebaceous glands that manifests with painful, inflammatory lesions in the apocrine glandular regions of the body, particularly the axillary, inguinal, and anogenital regions. Intensive clinical and experimental research and the review of new therapeutic approaches have almost completely renewed knowledge in the field of HS/AI since the publication of the old guideline in 2012. In Germany, a point prevalence of active disease of 0.3 % was observed, rising to 3.0 % when also taking into account patients with only scars typical of HS/AI. Pillars of HS/AI pathogenesis are abnormal differentiation of the keratinocytes of the pilosebaceous gland and massive accompanying inflammation. The primary lesions of HS/AI are inflammatory nodules, abscesses, and draining tunnels predominantly at predilection sites (axillary, submammary, inguinal, genital, and perineal). Recurrences in the last 6 months with at least 2 lesions at the sites of predilection indicate HS/AI. Although these criteria can be used to make a clinical diagnosis with a high accuracy of 97 %, the disease is still little known, as the delay in diagnosis in Germany of 10.0 ± 9.6 years proves. Patients with HS/AI suffer from a significant reduction in quality of life, especially when there is a high degree of morbidity. In daily practice, HS/AI should be classified and its activity assessed using a validated instrument, primarily using the International Hidradenitis Suppurativa Severity Scoring System (IHS4), in order to be able to make correct treatment decisions. HS/AI is divided into two forms related to the severity of the always detectable inflammation, namely the active, inflammatory and the inactive, predominantly non-inflammatory forms. While the intensity of the inflammatory form is divided into mild, moderate and severe HS/AI using the IHS4 classification and treated accordingly with medications, the predominantly non-inflammatory form is treated surgically according to the Hurley grade of the affected location, namely Hurley-Grade I, II and III. Oral tetracyclines or 5-day intravenous clindamycin therapy are equivalent to the effectiveness of the oral systemic combination of clindamycin and rifampicin. The subcutaneously administered monoclonal antibodies adalimumab and secukinumab are approved for the treatment of HS/AI. Various surgical procedures are available for the predominantly non-inflammatory form of the disease. The combination of drug therapy to reduce inflammation with a surgical procedure to eliminate irreversible tissue damage is currently considered a holistic therapy procedure for HS/AI. Regular monitoring and, if necessary, adjustment of the therapy with regard to a changing degree of disease severity is recommended.

Schlüsselwörter

Hidradenitis suppurativa - Acne inversa - Klassifikation - Schweregrad - Therapie - Leitlinie

Keywords

hidradenitis suppurativa - acne inversa - classification - severity - therapy - guideline

^* Koordination der Delegiertensitzung am 27.04.2023

Publication History

Article published online:
23 February 2024

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