Hidradenitis suppurativa

Zuletzt aktualisiert: 2025-02-10

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

ICD11: ED92.0

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: 

  • I Einzelne oder multiple Abszesse ohne Vernarbung oder Fisteln
  • II Rezidivierende Abszesse und Knoten mit Bildung von Fistelgängen oder Narben: Einzelne oder mehrere, weit voneinander entfernte Läsionen
  • III Diffuse oder nahezu diffuse Beteiligung mit mehreren miteinander verbundenen Fistelgängen, Narbenbildung und Abszessen im gesamten Areal

 

 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, Arthritis der kleinen und mittleren Gelenke
    • 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 tiefsitzender, kutan-subkutaner Knoten, der sich spontan zurückbildet, persistiert oder sich in einen Abszess umwandeln kann.
  • Abszesse können in der Tiefe verschmelzen, nach aussen spontan rupturierten 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.

  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. 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.
  4. 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.
  5. Arun, B. and A. Loffeld, Long-standing hidradenitis suppurativa treated effectively with metformin. Clin Exp Dermatol, 2009. 34(8): p. 920-1.
  6. Attanoos, R.L., et al., Granulomatous hidradenitis suppurativa and cutaneous Crohn's disease. Histopathology, 1993. 23(2): p. 111-115.
  7. 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.
  8. Bechara FG, Podda M, Prens EP. et al. Efficacy and Safety of Adalimumab in Conjunction with Surgery in Moderate to Severe Hidradenitis Suppurativa: The SHARPS Randomized Clinical Trial. JAMA Surg 2021; 156: 1001-1009.
  9. Bieniek A, Matusiak Ł, Okulewicz-Gojlik D, Szepietowski JC. Surgical Treatment of Hidradenitis Suppurativa: Experiences and Recommendations. Dermatologic Surgery. 2010;36:1998–2004.
  10. Bocchini, S.F., et al., Gluteal and Perianal Hidradenitis Suppurativa. Diseases of the Colon & Rectum, 2003. 46(7): p. 944-949.
  11. 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.
  12. 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.
  13. 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.
  14. Botvid SHC, Storgaard Hove L, Bouazzi D et al. Hidradenitis Suppurativa Prevalence in Nuuk, Greenland: Physician Validation of a Hidradenitis Suppurativa Questionnaire in a Greenlandic Setting. Acta Derm Venereol 2023; 103: adv00847 doi:10.2340/actadv.v103.4410.
  15. Brocard, A., et al., Hidradenitis suppurativa and zinc: a new therapeutic approach. A pilot study. Dermatology, 2007. 214(4): p. 325-7.
  16. Brunsting, H.A., HIDRADENITIS SUPPURATIVA; ABSCESS OF THE APOCRINE SWEAT GLANDS. Archives of Dermatology and Syphilology, 1939. 39(1): p. 108.
  17. Buckley, D.A. and S. Rogers, Cyclosporin-responsive hidradenitis suppurativa. J R Soc Med, 1995. 88(5): p. 289P-290P.
  18. Clemmensen, O.J., Topical treatment of hidradenitis suppurativa with clindamycin. Int J Dermatol, 1983. 22(5): p. 325-8.
  19. Cordain, L., et al., Acne vulgaris: a disease of Western civilization. Arch Dermatol, 2002. 138(12): p. 1584-90.
  20. Cusack, C. and C. Buckley, Etanercept: effective in the management of hidradenitis suppurativa. Br J Dermatol, 2006. 0(0): p. 060116091018022.
  21. Danby, F.W. and L.J. Margesson, Hidradenitis suppurativa. Dermatol Clin, 2010. 28(4): p. 779-93.
  22. 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.
  23. 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.
  24. 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.
  25. Dreno, B., et al., Hidradenitis suppurativa: the role of deficient cutaneous innate immunity. Arch Dermatol, 2012. 148(2): p. 182-6.
  26. 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.
  27. Fimmel, S. and C.C. Zouboulis, Comorbidities of hidradenitis suppurativa (acne inversa). Dermato-Endocrinology, 2010. 2(1): p. 9-16.
  28. 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.
  29. 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.
  30. 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.
  31. Gill L, Williams M, Hamzavi I. Update on hidradenitis suppurativa: connecting the tracts. F1000prime reports. 2014;6:112.
  32. 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.
  33. 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.
  34. 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.
  35. 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.
  36. 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.
  37. 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.
  38. Hsiao, J.L., et al., Hidradenitis Suppurativa and Concomitant Pyoderma Gangrenosum. Arch Dermatol, 2010. 146(11).
  39. Hunger RE, et al., Swiss Practice Recommendations for the Management of Hidradenitis Suppurativa/Acne Inversa.
  40. 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.
  41. Jemec GB. Clinical practice. Hidradenitis suppurativa. N Engl J Med. 2012;366:158–64.
  42. 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.
  43. Jemec, G.B., Clinical practice. Hidradenitis suppurativa. N Engl J Med, 2012. 366(2): p. 158-64.
  44. Jemec, G.B.E., Hidradenitis Suppurativa. Journal of Cutaneous Medicine and Surgery: Incorporating Medical and Surgical Dermatology, 2002. 7(1): p. 47-56.
  45. Jemec, G.B.E. and U. Hansen, Histology of hidradenitis suppurativa. Journal of the American Academy of Dermatology, 1996. 34(6): p. 994-999.
  46. Jfri A, Nassim D, O’Brien E. et al. Prevalence of Hidradenitis Suppurativa: A Systematic Review and Meta-regression Analysis. JAMA Dermatol 2021; 157: 924-931.
  47. Kagan, R.J., et al., Surgical treatment of hidradenitis suppurativa: A 10-year experience. Surgery, 2005. 138(4): p. 734-741.
  48. 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.
  49. 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.
  50. 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.
  51. Krueger, J.G. et al., Hidradenitis suppurativa: new insights into disease mechanisms and an evolving treatment landscape, British Journal of Dermatology, Volume 190, Issue 2, February 2024, Pages 149–162.
  52. Kurzen H, Kurokawa I, Jemec GBE et al. What causes hidradenitis suppurativa? Exp Dermatol 2008; 17: 455–456; discussion 457–472.
  53. 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.
  54. 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.
  55. Lavogiez, C., et al., Clinicopathological Study of 13 Cases of Squamous Cell Carcinoma Complicating Hidradenitis Suppurativa. Dermatology, 2010. 220(2): p. 147-153.
  56. 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.
  57. 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.
  58. Malaguarnera, M., et al., Squamous-cell cancer in Verneuil's disease (hidradenitis suppurativa). The Lancet, 1996. 348(9039): p. 1449.
  59. Martínez, F., et al., Hidradenitis Suppurativa and Crohn's Disease: Response to Treatment with Infliximab. Inflammatory Bowel Diseases, 2001. 7(4): p. 323-326.
  60. 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.
  61. 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.
  62. 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.
  63. 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.
  64. 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.
  65. Mendonca, C.O. and C.E. Griffiths, Clindamycin and rifampicin combination therapy for hidradenitis suppurativa. Br J Dermatol, 2006. 154(5): p. 977-8.
  66. Menis, D., et al., Two cases of severe hidradenitis suppurativa with failure of anakinra therapy. Br J Dermatol, 2015. 172(3): p. 810-1.
  67. 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.
  68. 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.
  69. Pérez-Diaz, D., et al., Squamous cell carcinoma complicating perianal hidradenitis suppurativa. Int J Colorect Dis, 1995. 10(4): p. 225-228.
  70. Ralf Paus, L., et al., What causes hidradenitis suppurativa? Experimental Dermatology, 2008. 17(5): p. 455-456.
  71. Revuz, J., Hidradenitis suppurativa. Journal of the European Academy of Dermatology and Venereology, 2009. 23(9): p. 985-998.
  72. 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.
  73. 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.
  74. 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.
  75. 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.
  76. 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.
  77. Sharon, V.R., et al., Management of recalcitrant hidradenitis suppurativa with ustekinumab. Acta Derm Venereol, 2012. 92(3): p. 320-1.
  78. 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.
  79. Soria, A., et al., Absence of Efficacy of Oral Isotretinoin in Hidradenitis Suppurativa: A Retrospective Study Based on Patients&rsquo; Outcome Assessment. Dermatology, 2009. 218(2): p. 134-135.
  80. 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.
  81. 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.
  82. 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.
  83. Tzellos T, van Straalen KR, Kyrgidis A. et al. Development and validation of IHS4-55, an IHS4 dichotomous outcome to assess treatment effect for hidradenitis suppurativa. J Eur Acad Dermatol Venereol 2023; 37: 395-401.
  84. 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.
  85. 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.
  86. 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.
  87. 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.
  88. van Straalen KR, Tzellos T, Guillem P. et al. The efficacy and tolerability of tetracyclines and clindamycin plus rifampicin for the treatment of hidradenitis suppurativa: Results of a prospective European cohort study. J Am Acad Dermatol 2021; 85: 369-378.
  89. 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.
  90. 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.
  91. 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.
  92. Zouboulis CC, Bechara FG, Dickinson-Blok JL. et al. Hidradenitis suppurativa/acne inversa: a practical framework for treatment optimization – systematic review and recommendations from the HS ALLIANCE working group. J Eur Acad Dermatol Venereol 2019; 33: 19-31.
  93. Zouboulis CC, Bechara FG, Fritz K, Wienert V, Winkler T. Leitlinien Acne Inversa / Hidradenitis suppurativa. AWMF. 2012.
  94. Zouboulis CC, Benhadou F, Byrd AS et al. What causes hidradenitis suppurativa? – 15 years after. Exp Dermatol 2020; 29: 1154–1170.
  95. 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: 184–190.
  96. 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.
  97. 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.
  98. Zouboulis, C.C., et al., S2k-Leitlinie zur Therapie der Hidradenitis suppurativa/Acne inversa.