Zusammenfassung
Die Hidradenitis suppurativa/Acne inversa (HS/AI) ist eine chronisch rezidivierende inflammatorische Hauterkrankung. Erhebungen zeigen, dass bevorzugt Männer einen perianalen, perinealen und glutealen Befall aufweisen, wohingegen bei Frauen die Axillen sowie die submammäre und inguinale Region vermehrt betroffen sind. Der Befall der Genitoanalregion ist mit einer stärkeren Einschränkung der Lebensqualität und einer größeren Belastung der sexuellen Gesundheit assoziiert als andere Lokalisationen. Darüber hinaus kann die HS/AI in der Genitoanalregion zu schwerwiegenden Komplikationen führen. Die perianale Fistelbildung, das pubogenitale Lymphödem und das Plattenepithelkarzinom zählen zu den 3 schwersten Komplikationen, deren Kenntnis entscheidend für ein adäquates Therapiemanagement ist.
Abstract
Hidradenitis suppurativa/acne inversa (HS/AI) is a chronic inflammatory skin disease. Several studies showed that perianal, perineal and gluteal involvement is more common in men. Axillary, submammary and inguinal localizations seem to be more prevalent in women. Involvement of the genitoanal region is associated with a higher reduced quality of live and sexual health compared to other locations. Moreover HS/AI in the genitoanal region can lead to serious complications. The knowledge of perianal fistula formation, pubogenital lymphedema and squamous cell carcinoma, which are three of the most severe complications, is critical for adequate treatment.
Literatur
Ardon CB, Molenaar C, van Straalen KR et al (2019) High prevalence of hidradenitis suppurativa in patients with perianal fistula. Int J Colorectal Dis 34:1337–1339. https://doi.org/10.1007/s00384-019-03313-2
Bechara FG (2016) MIBHS: a concept to distinguish between medical and surgical treatment in hidradenitis suppurativa. Exp Dermatol 25(Suppl):8. https://doi.org/10.1111/exd.12954
Boer J, Jemec GBE (2016) Mechanical stress and the development of pseudo-comedones and tunnels in Hidradenitis suppurativa/Acne inversa. Exp Dermatol 25:396–397. https://doi.org/10.1111/exd.12926
Chu EY, Kovarik CL, Lee RA (2013) Lymphedematous verrucous changes simulating squamous cell carcinoma in long-standing hidradenitis suppurativa. Int J Dermatol 52:808–812. https://doi.org/10.1111/j.1365-4632.2011.05361.x
Culp CE (1983) Chronic hidradenitis suppurativa of the anal canal. A surgical skin disease. Dis Colon Rectum 26:669–676. https://doi.org/10.1007/BF02553341
Frew JW, Vekic DA, Woods J, Cains GD (2017) A systematic review and critical evaluation of reported pathogenic sequence variants in hidradenitis suppurativa. Br J Dermatol 177:987–998. https://doi.org/10.1111/bjd.15441
Gooderham M, Papp K (2015) The psychosocial impact of hidradenitis suppurativa. J Am Acad Dermatol 73:S19–S22. https://doi.org/10.1016/j.jaad.2015.07.054
Gulliver W, Zouboulis CC, Prens E et al (2016) Evidence-based approach to the treatment of hidradenitis suppurativa/acne inversa, based on the European guidelines for hidradenitis suppurativa. Rev Endocr Metab Disord 29:619–644. https://doi.org/10.1007/s11154-016-9328-5
Hessam S, Gambichler T, Höxtermann S et al (2019) Frequency of circulating subpopulations of T regulatory cells in patients with hidradenitis suppurativa. J Eur Acad Dermatol Venereol. https://doi.org/10.1111/jdv.16071
Hessam S, Sand M, Bechara FG (2017) When inflammation shifts to malignancy: extensive squamous cell carcinoma in a female hidradenitis suppurativa/acne inversa patient. J Dtsch Dermatol Ges 15:86–88. https://doi.org/10.1111/ddg.12911
Hessam S, Sand M, Gambichler T et al (2018) Interleukin-36 in hidradenitis suppurativa: evidence for a distinctive proinflammatory role and a key factor in the development of an inflammatory loop. Br J Dermatol 178:761–767. https://doi.org/10.1111/bjd.16019
Hessam S, Sand M, Gambichler T, Bechara FG (2015) Correlation of inflammatory serum markers with disease severity in patients with hidradenitis suppurativa (HS). J Am Acad Dermatol 73:998–1005. https://doi.org/10.1016/j.jaad.2015.08.052
Hessam S, Sand M, Lang K et al (2017) Altered global 5‑Hydroxymethylation status in Hidradenitis Suppurativa: support for an Epigenetic background. Dermatology 233:129–135. https://doi.org/10.1159/000478043
Hessam S, Sand M, Skrygan M et al (2016) Inflammation induced changes in the expression levels of components of the microRNA maturation machinery Drosha, Dicer, Drosha co-factor DGRC8 and Exportin‑5 in inflammatory lesions of hidradenitis suppurativa patients. J Dermatol Sci 82:166–174. https://doi.org/10.1016/j.jdermsci.2016.02.009
Hessam S, Sand M, Skrygan M et al (2017) Expression of miRNA-155, miRNA-223, miRNA-31, miRNA-21, miRNA-125b, and miRNA-146a in the inflammatory pathway of Hidradenitis Suppurativa. Inflammation 40:464–472. https://doi.org/10.1007/s10753-016-0492-2
Hessam S, Sand M, Skrygan M, Bechara FG (2017) The microRNA effector RNA-induced silencing complex in hidradenitis suppurativa: a significant dysregulation within active inflammatory lesions. Arch Dermatol Res 309:557–565. https://doi.org/10.1007/s00403-017-1752-1
Hessam S, Scholl L, Sand M et al (2018) A novel severity assessment scoring system for Hidradenitis Suppurativa. JAMA Dermatol 154:330–335. https://doi.org/10.1001/jamadermatol.2017.5890
Hokkanen SR, Boxall N, Khalid JM et al (2019) Prevalence of anal fistula in the United Kingdom. World J Clin Cases 7:1795–1804. https://doi.org/10.12998/wjcc.v7.i14.1795
Horváth B, Janse I, Blok J et al (2017) Hurley staging refined: a proposal by the Dutch Hidradenitis Suppurativa expert group. Acta Derm Venereol 97:412–413. https://doi.org/10.2340/00015555-2513
Hurley H (1996) Axillary hyperhidrosis, apocrine bromhidrosis, hidradenitis suppurativa and familial benign pemphigus. Surgical approach. In: Roenigk R, Roenigk H (Hrsg) Roenigk & Roenigk’s Dermatologic surgery: principles and practice, 2nd. Aufl. Marcel Dekker, New York, S 623–664
Kimball AB, Okun MM, Williams DA et al (2016) Two phase 3 trials of Adalimumab for Hidradenitis Suppurativa. N Engl J Med 375:422–434. https://doi.org/10.1056/NEJMoa1504370
Kirschke J, Hessam S, Bechara FG (2015) Hidradenitis suppurativa/acne inversa: Ein Update. Hautarzt 66:413–422. https://doi.org/10.1007/s00105-015-3616-y
Kohorst JJ, Shah KK, Hallemeier CL et al (2019) Squamous cell carcinoma in perineal, Perianal, and gluteal Hidradenitis Suppurativa: experience in 12 patients. Dermatol Surg 45:519–526. https://doi.org/10.1097/DSS.0000000000001713
Kurek A, Peters EMJ, Chanwangpong A et al (2012) Profound disturbances of sexual health in patients with acne inversa. J Am Acad Dermatol 67:422–428.e1. https://doi.org/10.1016/j.jaad.2011.10.024
Matusiak L, Bieniek A, Szepietowski JC (2010) Psychophysical aspects of hidradenitis suppurativa. Acta Derm Venereol 90:264–268. https://doi.org/10.2340/00015555-0866
Micieli R, Alavi A (2018) Lymphedema in patients with hidradenitis suppurativa: a systematic review of published literature. Int J Dermatol 57:1471–1480. https://doi.org/10.1111/ijd.14173
Musumeci ML, Scilletta A, Sorci F et al (2019) Genital lymphedema associated with hidradenitis suppurativa unresponsive to adalimumab treatment. JAAD Case Rep 5:326–328. https://doi.org/10.1016/j.jdcr.2019.01.019
Patino G, Zheng MY, Breyer BN, Cohen AJ (2019) Skin grafting applications in urology. Rev Urol 21:8–14
Revuz J (2009) Hidradenitis suppurativa. J Eur Acad Dermatol Venereol 23:985–998. https://doi.org/10.1111/j.1468-3083.2009.03356.x
Sabat R, Tsaousi A, Rossbacher J et al (2017) Acne inversa/Hidradenitis suppurativa: Ein Update. Hautarzt 68:999–1006. https://doi.org/10.1007/s00105-017-4082-5
Saunte DM, Boer J, Stratigos A et al (2015) Diagnostic delay in hidradenitis suppurativa is a global problem. Br J Dermatol 173:1546–1549. https://doi.org/10.1111/bjd.14038
Schneider-Burrus S, Arpa E, Kors C et al (2018) Medikamentöse Therapie der Acne inversa. Hautarzt 69:58–63. https://doi.org/10.1007/s00105-017-4094-1
Scholl L, Hessam S, Bergmann U, Bechara FG (2018) Surgical treatment of sinus tracts and fistulas in Perianal Hidradenitis Suppurativa. J Cutan Med Surg 22:239–241. https://doi.org/10.1177/1203475417746122
Scholl L, Hessam S, Reitenbach S, Bechara FG (2018) Operative Behandlungsoptionen bei Hidradenitis suppurativa/Acne inversa. Hautarzt 69:149–161. https://doi.org/10.1007/s00105-017-4114-1
Shanmugam VK, Mulani S, McNish S et al (2018) Longitudinal observational study of hidradenitis suppurativa: impact of surgical intervention with adjunctive biologic therapy. Int J Dermatol 57:62–69. https://doi.org/10.1111/ijd.13798
Zouboulis CC, Bechara FG, Fritz K et al (2012) S1-Leitlinie zur Therapie der Hidradenitis suppurativa/Acne inversa. J Dtsch Dermatol Ges 10(Suppl 5):S1–S31. https://doi.org/10.1111/j.1610-0387.2012.08006.x
Zouboulis CC, Desai N, Emtestam L et al (2015) European S1 guideline for the treatment of hidradenitis suppurativa/acne inversa. J Eur Acad Dermatol Venereol 29:619–644. https://doi.org/10.1111/jdv.12966
Zouboulis CC, Okun MM, Prens EP et al (2019) Long-term adalimumab efficacy in patients with moderate-to-severe hidradenitis suppurativa/acne inversa: 3‑year results of a phase 3 open-label extension study. J Am Acad Dermatol 80:60–69.e2. https://doi.org/10.1016/j.jaad.2018.05.040
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S. Hessam hat als stellvertretender Studienleiter von der Firma AbbVie gesponserte Phase-III- und -IV-Studien betreut und als Vortragender finanzielle Zuwendung sowie Reisekostenunterstützung erhalten. F.G. Bechara hat als Vortragender und Studienleiter finanzielle Zuwendungen sowie Reisekostenunterstützung von der Firma AbbVie erhalten.
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Hessam, S., Bechara, F.G. Hidradenitis suppurativa/Acne inversa in der Genitoanalregion. Hautarzt 71, 256–262 (2020). https://doi.org/10.1007/s00105-020-04556-9
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DOI: https://doi.org/10.1007/s00105-020-04556-9