Hidradenitis suppurativa

Letztmals aufdatiert: 2021-10-15

Autor(en): Anzengruber, Navarini

Verneuil 1854, Plewig & Steger 1989 

Akne inversa

Chronische-rezidiverende Entzündung der Haarfollikel (Terminalhaare) in den intertriginösen Areale mit Komedo-, Abszess-, Fistel- und Narbenstrangbildung. Keine primäre Schweissdrüsen-Entzündung, hingegen sind die apokrinen Drüsen (mit-)betroffen. 

  • 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
  • Ungeklärt
  • Assoziationen
    • Morbus Crohn
    • Rheumafaktor-negative Polyarthritiden
    • Hypertonie
    • Pyoderma gangraenosum
    • Systemische Amyloidose
  • Prädispositionsfaktoren:
    • Nikotinkonsum
    • Übergewicht
    • Enge Kleidung
  • Auftreten von 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
  • Rezidivierende Abszesse in den grossen Körperfalten
  • Vorhandensein von Risikofaktoren kombiniert mit der Klinik
  • Restliche Akne-Tetrade

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).

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

  • Rezidiv
    • 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 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.

USB Dermatologie Standard:

Topische Therapie (immer topisch behandeln, genügt allerdings nur bei Grad I und mild ausgeprägtem Grad II): 

  • Clindamycin Emulsion 2x tgl. für 3 Monate
    • Zur Vermeidung von Resistenzen immer gemeinsame Rezeptierung mit Triclosan Hautwaschlotion 1x tgl.
  • Triamcinolon Kenacort A40 1:1 Lidocain mit Luer lock 100uL in die Knoten spritzen, Achtung Atrophiegefahr aufklären

Systemische Therapie

  • Doxycyclin p.o. 100 mg 2x tgl.  über 3 Monate, kann nach 4 Wochen bei gutem Ansprechen reduziert werden auf 40mg tgl. (Oracea)
  • Rifampicin & Clindamycin
    • Clindamycin p.o. 300 mg 2x tgl. und Rifampicin p.o. 300 mg 2x tgl. über 3 Monate (nebenwirkungsreich und kaum nachhaltig)
  • TNF-alpha-Inhibitoren
    • Adalimumab s.c. 160 mg (Woche 0), 80 mg (Woche 1), 80 mg 1x wöchentlich
    • Certolizumab bei gebärfähigen Frauen (bis jetzt noch off-label)
  • Antiandrogene Therapie

Weitere Therapien

  • Infliximab i.v. 5 mg kg KG Woche 0, 2, 6 und danach alle 8 Wochen
  • Zinkgluconat p.o. 90 mg 1x tgl. für 3 Monate
  • Acitretin
  • Metformin

Isotretinoin ist NICHT indiziert!

Chirurgische Exzision

  • Kann in allen 3 Stadien durchgeführt werden.
  • Deroofing von Abszessen mittels Punchbiopsie (6er) kann zur effektiven Drainage und Abheilung führen
  • Ist rezidivarm
  • Weite Exzision und Sekundärheilung kann zu monatelanger Abheilungsdauer führen, am Spital nicht mehr machbar

Laser

  • CO2 Laser zur Ablation von Einzelherden funktioniert sehr gut
  • Haar-Entfernungslaser-Optionen
  1. Adams, D.R., et al., Treatment of Hidradenitis Suppurativa With Etanercept Injection. Arch Dermatol, 2010. 146(5).
  2. Alikhan, A., P.J. Lynch, and D.B. Eisen, Hidradenitis suppurativa: A comprehensive review. Journal of the American Academy of Dermatology, 2009. 60(4): p. 539-561.
  3. Attanoos, R.L., et al., Granulomatous hidradenitis suppurativa and cutaneous Crohn's disease. Histopathology, 1993. 23(2): p. 111-115.
  4. Barth, J.H. and T. Kealey, Androgen metabolism by isolated human axillary apocrine glands in hidradenitis suppurativa. Br J Dermatol, 1991. 125(4): p. 304-308.
  5. Bocchini, S.F., et al., Gluteal and Perianal Hidradenitis Suppurativa. Diseases of the Colon & Rectum, 2003. 46(7): p. 944-949.
  6. Brunsting, H.A., HIDRADENITIS SUPPURATIVA; ABSCESS OF THE APOCRINE SWEAT GLANDS. Archives of Dermatology and Syphilology, 1939. 39(1): p. 108.
  7. Cusack, C. and C. Buckley, Etanercept: effective in the management of hidradenitis suppurativa. Br J Dermatol, 2006. 0(0): p. 060116091018022.
  8. Fimmel, S. and C.C. Zouboulis, Comorbidities of hidradenitis suppurativa (acne inversa). Dermato-Endocrinology, 2010. 2(1): p. 9-16.
  9. Finley, E.M. and J.L. Ratz, Treatment of hidradenitis suppurativa with carbon dioxide laser excision and second-intention healing. Journal of the American Academy of Dermatology, 1996. 34(3): p. 465-469.
  10. Gener, G., et al., Combination Therapy with Clindamycin and Rifampicin for Hidradenitis Suppurativa: A Series of 116 Consecutive Patients. Dermatology, 2009. 219(2): p. 148-154.
  11. Giamarellos-Bourboulis, E.J., et al., An open-label phase II study of the safety and efficacy of etanercept for the therapy of hidradenitis suppurativa. British Journal of Dermatology, 2007. 158(3): p. 567-572.
  12. Haslund, P., R.A. Lee, and G.B.E. Jemec, Treatment of Hidradenitis Suppurativa with Tumour Necrosis Factor-alpha Inhibitors. Acta Dermato Venereologica, 2009. 89(6): p. 595-600.
  13. Hsiao, J.L., et al., Hidradenitis Suppurativa and Concomitant Pyoderma Gangrenosum. Arch Dermatol, 2010. 146(11).
  14. Jansen, T. and G. Plewig, What's new in acne inversa (alias hidradenitis suppurativa)? Journal of the European Academy of Dermatology and Venereology, 2000. 14(5): p. 342-343.
  15. Jemec, G.B.E., Hidradenitis Suppurativa. Journal of Cutaneous Medicine and Surgery: Incorporating Medical and Surgical Dermatology, 2002. 7(1): p. 47-56.
  16. Jemec, G.B.E. and U. Hansen, Histology of hidradenitis suppurativa. Journal of the American Academy of Dermatology, 1996. 34(6): p. 994-999.
  17. Kagan, R.J., et al., Surgical treatment of hidradenitis suppurativa: A 10-year experience. Surgery, 2005. 138(4): p. 734-741.
  18. Katsanos, K.H., D.K. Christodoulou, and E.V. Tsianos, Axillary hidradenitis suppurativa successfully treated with infliximab in a Crohn's disease patient. The American Journal of Gastroenterology, 2002. 97(8): p. 2155-2156.
  19. Lapins, J., J.A. Marcusson, and L. Emtestam, Surgical treatment of chronic hidradenitis suppurativa: CO2 laser stripping-secondary intention technique. British Journal of Dermatology, 2006. 131(4): p. 551-556.
  20. Lapins, J., O. Olerup, and L. Emtestam, No Human Leukocyte Antigen-A, -B or -DR Association in Swedish Patients with Hidradenitis Suppurativa. Acta Dermato-Venereologica, 2001. 81(1): p. 28-30.
  21. Lavogiez, C., et al., Clinicopathological Study of 13 Cases of Squamous Cell Carcinoma Complicating Hidradenitis Suppurativa. Dermatology, 2010. 220(2): p. 147-153.
  22. Malaguarnera, M., et al., Squamous-cell cancer in Verneuil's disease (hidradenitis suppurativa). The Lancet, 1996. 348(9039): p. 1449.
  23. Martínez, F., et al., Hidradenitis Suppurativa and Crohn's Disease: Response to Treatment with Infliximab. Inflammatory Bowel Diseases, 2001. 7(4): p. 323-326.
  24. Matusiak, L., A. Bieniek, and J.C. Szepietowski, Increased Serum Tumour Necrosis Factor- in Hidradenitis Suppurativa Patients: Is There a Basis for Treatment with Anti-Tumour Necrosis Factor- Agents? Acta Dermato Venereologica, 2009. 89(6): p. 601-603.
  25. Matusiak, Ł., A. Bieniek, and J.C. Szepietowski, Soluble interleukin-2 receptor serum level is a useful marker of hidradenitis suppurativa clinical staging. Biomarkers, 2009. 14(6): p. 432-437.
  26. Mekkes, J.R. and J.D. Bos, Long-term efficacy of a single course of infliximab in hidradenitis suppurativa. British Journal of Dermatology, 2007. 158(2): p. 370-374.
  27. Pérez-Diaz, D., et al., Squamous cell carcinoma complicating perianal hidradenitis suppurativa. Int J Colorect Dis, 1995. 10(4): p. 225-228.
  28. Ralf Paus, L., et al., What causes hidradenitis suppurativa? Experimental Dermatology, 2008. 17(5): p. 455-456.
  29. Revuz, J., Hidradenitis suppurativa. Journal of the European Academy of Dermatology and Venereology, 2009. 23(9): p. 985-998.
  30. Revuz, J.E., et al., Prevalence and factors associated with hidradenitis suppurativa: Results from two case-control studies. Journal of the American Academy of Dermatology, 2008. 59(4): p. 596-601.
  31. Rose, R.F., M.J.D. Goodfield, and S.M. Clark, Treatment of recalcitrant hidradenitis suppurativa with oral ciclosporin. Clin Exp Dermatol, 2006. 31(1): p. 154-155.
  32. Roy, M.K., et al., Probable association between hidradenitis suppurativa and Crohn's disease: Significance of epithelioid granuloma. British Journal of Surgery, 1997. 84(3): p. 375-376.
  33. Sartorius, K., et al., Objective scoring of hidradenitis suppurativa reflecting the role of tobacco smoking and obesity. British Journal of Dermatology, 2009. 161(4): p. 831-839.
  34. Sartorius, K., et al., Suggestions for uniform outcome variables when reporting treatment effects in hidradenitis suppurativa. Br J Dermatol, 2003. 149(1): p. 211-213.
  35. Slade, D.E.M., B.W. Powell, and P.S. Mortimer, Hidradenitis suppurativa: pathogenesis and management. British Journal of Plastic Surgery, 2003. 56(5): p. 451-461.
  36. Soria, A., et al., Absence of Efficacy of Oral Isotretinoin in Hidradenitis Suppurativa: A Retrospective Study Based on Patients’ Outcome Assessment. Dermatology, 2009. 218(2): p. 134-135.
  37. Sparks, M.K., Hypercalcemia in association with cutaneous squamous cell carcinoma. Occurrence as a late complication of hidradenitis suppurativa. Archives of Dermatology, 1985. 121(2): p. 243-246.
  38. Von Der Werth, J.M., H.C. Williams, and J.A. Raeburn, The clinical genetics of hidradenitis suppurativa revisited. British Journal of Dermatology, 2000. 142(5): p. 947-953.
  39. Jemec GB. Clinical practice. Hidradenitis suppurativa. N Engl J Med. 2012;366:158–64.
  40. Gill L, Williams M, Hamzavi I. Update on hidradenitis suppurativa: connecting the tracts. F1000prime reports. 2014;6:112.
  41. Zouboulis CC, Bechara FG, Fritz K, Wienert V, Winkler T. Leitlinien Acne Inversa / Hidradenitis suppurativa. AWMF. 2012.
  42. Zouboulis CC, Del Marmol V, Mrowietz U, et al. Hidradenitis Suppurativa/Acne Inversa: Criteria for Diagnosis, Severity Assessment, Classification and Disease Evaluation. Dermatology. 2015;231:184–90.
  43. Bieniek A, Matusiak Ł, Okulewicz-Gojlik D, Szepietowski JC. Surgical Treatment of Hidradenitis Suppurativa: Experiences and Recommendations. Dermatologic Surgery. 2010;36:1998–2004.
  44. Boer J, Jemec GBE. Resorcinol peels as a possible self-treatment of painful nodules in hidradenitis suppurativa. Clinical and experimental dermatology. 2010;35:36–40.
  45. Kimball AB, Szapary P, Mrowietz U, et al. Underdiagnosis and undertreatment of cardiovascular risk factors in patients with moderate to severe psoriasis. Journal of the American Academy of Dermatology. 2012;67:76–85.
  46. Miller I, Lynggaard CD, Lophaven S, Zachariae C, Dufour DN, Jemec GBE. A double-blind placebo-controlled randomized trial of adalimumab in the treatment of hidradenitis suppurativa. British Journal of Dermatology. 2011;165:391–8.
  47. Van Rappard DC, Mekkes JR. Treatment of severe hidradenitis suppurativa with infliximab in combination with surgical interventions. British Journal of Dermatology. 2012;167:206–8.
  48. Jemec, G.B., Clinical practice. Hidradenitis suppurativa. N Engl J Med, 2012. 366(2): p. 158-64.
  49. Cordain, L., et al., Acne vulgaris: a disease of Western civilization. Arch Dermatol, 2002. 138(12): p. 1584-90.
  50. Melnik, B.C., Evidence for acne-promoting effects of milk and other insulinotropic dairy products. Nestle Nutr Workshop Ser Pediatr Program, 2011. 67: p. 131-45.
  51. Danby, F.W. and L.J. Margesson, Hidradenitis suppurativa. Dermatol Clin, 2010. 28(4): p. 779-93.
  52. Di Landro, A., et al., Family history, body mass index, selected dietary factors, menstrual history, and risk of moderate to severe acne in adolescents and young adults. J Am Acad Dermatol, 2012. 67(6): p. 1129-35.
  53. Melnik, B.C., S.M. John, and G. Schmitz, Over-stimulation of insulin/IGF-1 signaling by western diet may promote diseases of civilization: lessons learnt from laron syndrome. Nutr Metab (Lond), 2011. 8: p. 41.
  54. Clemmensen, O.J., Topical treatment of hidradenitis suppurativa with clindamycin. Int J Dermatol, 1983. 22(5): p. 325-8.
  55. Jemec, G.B. and P. Wendelboe, Topical clindamycin versus systemic tetracycline in the treatment of hidradenitis suppurativa. J Am Acad Dermatol, 1998. 39(6): p. 971-4.
  56. Revuz, J., Hidradenitis suppurativa. J Eur Acad Dermatol Venereol, 2009. 23(9): p. 985-98.
  57. van der Zee, H.H., et al., The effect of combined treatment with oral clindamycin and oral rifampicin in patients with hidradenitis suppurativa. Dermatology, 2009. 219(2): p. 143-7.
  58. Mendonca, C.O. and C.E. Griffiths, Clindamycin and rifampicin combination therapy for hidradenitis suppurativa. Br J Dermatol, 2006. 154(5): p. 977-8.
  59. Grant, A., et al., Infliximab therapy for patients with moderate to severe hidradenitis suppurativa: a randomized, double-blind, placebo-controlled crossover trial. J Am Acad Dermatol, 2010. 62(2): p. 205-17.
  60. Lesage, C., et al., Efficacy and tolerance of prolonged infliximab treatment of moderate-to-severe forms of hidradenitis suppurativa. Eur J Dermatol, 2012. 22(5): p. 640-4.
  61. Delage, M., et al., Efficacy of infliximab for hidradenitis suppurativa: assessment of clinical and biological inflammatory markers. Acta Derm Venereol, 2011. 91(2): p. 169-71.
  62. Kimball, A.B., et al., Adalimumab for the treatment of moderate to severe Hidradenitis suppurativa: a parallel randomized trial. Ann Intern Med, 2012. 157(12): p. 846-55.
  63. van Rappard, D.C., et al., Comparing treatment outcome of infliximab and adalimumab in patients with severe hidradenitis suppurativa. J Dermatolog Treat, 2012. 23(4): p. 284-9.
  64. Amano, M., A. Grant, and F.A. Kerdel, A prospective open-label clinical trial of adalimumab for the treatment of hidradenitis suppurativa. Int J Dermatol, 2010. 49(8): p. 950-5.
  65. Arenbergerova, M., S. Gkalpakiotis, and P. Arenberger, Effective long-term control of refractory hidradenitis suppurativa with adalimumab after failure of conventional therapy. Int J Dermatol, 2010. 49(12): p. 1445-9.
  66. Sotiriou, E., et al., A prospective open-label clinical trial of efficacy of the every week administration of adalimumab in the treatment of hidradenitis suppurativa. J Drugs Dermatol, 2012. 11(5 Suppl): p. s15-20.
  67. Pelekanou, A., et al., Long-term efficacy of etanercept in hidradenitis suppurativa: results from an open-label phase II prospective trial. Exp Dermatol, 2010. 19(6): p. 538-40.
  68. Gulliver, W.P., G.B. Jemec, and K.A. Baker, Experience with ustekinumab for the treatment of moderate to severe hidradenitis suppurativa. J Eur Acad Dermatol Venereol, 2012. 26(7): p. 911-4.
  69. Sharon, V.R., et al., Management of recalcitrant hidradenitis suppurativa with ustekinumab. Acta Derm Venereol, 2012. 92(3): p. 320-1.
  70. Tzanetakou, V., et al., Safety and Efficacy of Anakinra in Severe Hidradenitis Suppurativa: A Randomized Clinical Trial. JAMA Dermatol, 2016. 152(1): p. 52-9.
  71. Zarchi, K., D.N. Dufour, and G.B. Jemec, Successful treatment of severe hidradenitis suppurativa with anakinra. JAMA Dermatol, 2013. 149(10): p. 1192-4.
  72. Leslie, K.S., et al., An open-label study of anakinra for the treatment of moderate to severe hidradenitis suppurativa. J Am Acad Dermatol, 2014. 70(2): p. 243-51.
  73. van der Zee, H.H. and E.P. Prens, Failure of anti-interleukin-1 therapy in severe hidradenitis suppurativa: a case report. Dermatology, 2013. 226(2): p. 97-100.
  74. Menis, D., et al., Two cases of severe hidradenitis suppurativa with failure of anakinra therapy. Br J Dermatol, 2015. 172(3): p. 810-1.
  75. Boer, J. and M. Nazary, Long-term results of acitretin therapy for hidradenitis suppurativa. Is acne inversa also a misnomer? Br J Dermatol, 2011. 164(1): p. 170-5.
  76. Boer, J. and M.J. van Gemert, Long-term results of isotretinoin in the treatment of 68 patients with hidradenitis suppurativa. J Am Acad Dermatol, 1999. 40(1): p. 73-6.
  77. Gupta, A.K., et al., Oral cyclosporine in the treatment of inflammatory and noninflammatory dermatoses. A clinical and immunopathologic analysis. Arch Dermatol, 1990. 126(3): p. 339-50.
  78. Buckley, D.A. and S. Rogers, Cyclosporin-responsive hidradenitis suppurativa. J R Soc Med, 1995. 88(5): p. 289P-290P.
  79. Ducroux, E., et al., Hidradenitis suppurativa after renal transplantation: complete remission after switching from oral cyclosporine to oral tacrolimus. J Am Acad Dermatol, 2014. 71(5): p. e210-1.
  80. Verdolini, R., et al., Metformin for the treatment of hidradenitis suppurativa: a little help along the way. J Eur Acad Dermatol Venereol, 2013. 27(9): p. 1101-8.
  81. Arun, B. and A. Loffeld, Long-standing hidradenitis suppurativa treated effectively with metformin. Clin Exp Dermatol, 2009. 34(8): p. 920-1.
  82. Brocard, A., et al., Hidradenitis suppurativa and zinc: a new therapeutic approach. A pilot study. Dermatology, 2007. 214(4): p. 325-7.
  83. Dreno, B., et al., Hidradenitis suppurativa: the role of deficient cutaneous innate immunity. Arch Dermatol, 2012. 148(2): p. 182-6.
  84. Deckers, I.E., et al., Fumarates, a new treatment option for therapy-resistant hidradenitis suppurativa: a prospective open-label pilot study. Br J Dermatol, 2015. 172(3): p. 828-9.
  85. Guillet, A., et al., Verneuil's disease, innate immunity and vitamin D: a pilot study. J Eur Acad Dermatol Venereol, 2015. 29(7): p. 1347-53.
  86. Gulliver, W., et al., Evidence-based approach to the treatment of hidradenitis suppurativa/acne inversa, based on the European guidelines for hidradenitis suppurativa. Rev Endocr Metab Disord, 2016.
  87. Zouboulis, C.C., et al., European S1 guideline for the treatment of hidradenitis suppurativa/acne inversa. J Eur Acad Dermatol Venereol, 2015. 29(4): p. 619-44.