Hidradenitis Suppurativa: Modern Concepts of Pathogenesis and Therapy; Successful Experience of Adalimumab Therapy

Cover Page


Cite item

Full Text

Abstract

Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease that mainly affects the sites of the apocrine glands; HS is characterised by relapsing painful knots and abscesses, leading to the formation of fistulas and scarring. Modern ideas about the pathogenesis of HS allow us to consider it as a kind of inverse acne, since the mechanism of development of HS and inverse acne does not differ. Detailed studies of patients with HS in recent years have provided the basis for several authors to suggest that HS is a systemic, chronic, immune-mediated progressive disease. The key role of the proinflammatory cytokine tumor necrosis factor Į (TNF-Į) in the development of the inflammatory process during HS was determined. Elevated TNF-Į levels are found in the blood and HS lesions, having a positive correlation of the TNF-Į level with the severity of the disease. At the present time, the treatment with a TNF-Į blocker, adalimumab (the only one genetically engineered biological preparation approved for treatment of HS/inverse acne in the world and in Russia), is recommended as a first-line therapy in the absence of an adequate response to standard systemic therapy in the case of active HS of moderate or utmost severity. The presented clinical case confirms the fact that for HS patients it is very important to determine the correct diagnosis as quickly as possible and to prescribe the appropriate treatment according to the developed recommendations. Erroneous diagnoses, inadequate therapy (surgical treatment, short courses of antibiotics, etc.) contribute to the progression of the disease, to the appearance of common symptoms due to the attachment of secondary infection and increase in general intoxication, to limited working capacity, to the emergence of persistent and gross scars, as well as to the deformations of affected skin. Our clinical case of adalimumab (Humira) treatment of a patient with an extremely severe HS form demonstrated that the application of this medication can be effective and well tolerated. 

About the authors

M. M. Khobeish

Academician I. P. Pavlov First St. Petersburg State Medical University of the Ministry of Health of Russian Federation, St. Petersburg

Author for correspondence.
Email: mkhobeysh@yandex.ru
L'va Tolstogo str., 6/8, bldg 4, 197022 Россия

D. V. Shustov

Academician I. P. Pavlov First St. Petersburg State Medical University of the Ministry of Health of Russian Federation, St. Petersburg

Email: fake@neicon.ru
L'va Tolstogo str., 6/8, bldg 4, 197022 Россия

E. V. Sokolovsky

Academician I. P. Pavlov First St. Petersburg State Medical University of the Ministry of Health of Russian Federation, St. Petersburg

Email: fake@neicon.ru
L'va Tolstogo str., 6/8, bldg 4, 197022 Россия

References

  1. van der Zee H. H., Laman J. D., Boer J., Prens E. P. Hidradenitis Suppurativa: Viewpoint on Clinical Phenotyping, Pathogenesis and Novel Treatments. Exp. Dermatol. 2012;21(10):735–739.
  2. Jemec G. B. Clinical Practice: Hidradenitis Suppurativa. N. Engl. J. Med. 2012;366(2):158–164.
  3. Михеев Г. Н., Аравийская Е. Р., Соколовский Е. В., Холо- дилова Н. А. Инверсные акне: современные представления о патогенезе, клинических проявлениях и терапии. Пластическая хирургия и косметология. 2010;(3):433–438. [Mikheev G. N., Araviiskaya E. R., Sokolovskiy E. V., Kholodilova N. A. Inverse Acne: Modern Concepts of Pathogenesis, Clinical Manifestations and Therapy. Plastic Surgery and Cosmetology. 2010;(3):433–438.]
  4. Poli F., Jemec G. B., Revuz J. Clinical Presentation. In: Hidradenitis Suppurativa. Ed. by G. B. Jemec, J. Revuz, J. J. Leyden. Heidelberg: Springer-Verlag, 2006. P. 11–24.
  5. van der Zee H. H., de Ruiter L., van den Broecke D. G. et al. Elevated Levels of Tumour Necrosis Factor (TNF)-Į, Interleukin (IL)-1ȕ and IL-10 in Hidradenitis Suppurativa Skin: a Rationale for Targeting TNF-Į and IL-1ȕ. Br. J. Dermatol. 2011;164(6):1292–1298.
  6. Giamarellos-Bourboulis E. J., Antonopoulou A., Petropoulou C. et al. Altered Innate and Adaptive Immune Responses in Patients with Hidradenitis Suppurativa. Br. J. Dermatol. 2007;156(1):51–56.
  7. Napolitano M., Megna M., Timoshchuk E. A. et al. Hidradenitis Suppurativa: from Pathogenesis to Diagnosis and Treatment. Clin., Cosmet. Investig. Dermatol. 2017;10:105–115.
  8. Collier F., Smith R. C., Morton C. A. Diagnosis and Management of Hidradenitis Suppurativa. BMJ. 2013;346:f2121.
  9. Matusiak L., Bieniek A., Szepietowski J. C. Increased Serum Tumour Necrosis Factor-Alpha in Hidradenitis Suppurativa Patients: is There a Basis for Treatment with Anti-Tumour Necrosis Factor-Alpha Agents? Acta Derm. Venereol. 2009;89(6):601–603.
  10. Mozeika E., Pilmane M., Nürnberg B. M., Jemec G. B. Tumour Necrosis Factor-Alpha and Matrix Metalloproteinase-2 are Expressed Strongly in Hidradenitis Suppurativa. Acta Derm. Venereol. 2013;93(3):301–304.
  11. Humira (Summary of Product Characteristics). AbbVie Ltd., 2015.
  12. Nazary M., van der Zee H. H., Prens E. P. et al. Pathogenesis and Pharmacotherapy of hidratenitis Suppurativa. Eur. J. Pharmacol. 2011;672(1– 3):1–8.
  13. Weller R., Hunter J., Savin J. et al. The Function and Structure of the Skin. In: Clinical Dermatology. Ed. by M. Sugden, R. Blundell. 4th ed. Malden, MA: Blackwell Publishing, Inc., 2008. P. 10–33.
  14. Fitzsimmons J. S., Guilbert P. R. A Family Study of Hidradenitis Suppurativa. J. Med. Genet. 1985;22(5):367–373.
  15. Pink A. E., Simpson M. A., Desai N. et al. Mutations in the Ȗ-Secretase Genes NCSTN, PSENEN, and PSEN1 Underlie Rare Forms of Hidradenitis Suppurativa (Acne Inversa). J. Invest. Dermatol. 2012;132(10):2459–2461.
  16. Jemec G. B., Heidenheim M., Nielsen N. H. The Prevalence of Hidradenitis Suppurativa and Its Potential Precursor Lesions. J. Am. Acad. Dermatol. 1996;35(2 Pt 1):191–194.
  17. Revuz J. E., Canoui-Poitrine F., Pier Wolkenstein et al. Prevalence and Factors Associated with Hidradenitis Suppurativa: Results from Two Case-Control Studies. J. Am. Acad. Dermatol. 2008;59(4):596–601.
  18. Jemec G. B. The Symptomatology of Hidradenitis Suppurativa in Women. Br. J. Dermatol. 1988;119(3):345–350.
  19. Vazquez B. G., Alikhan A., Weaver A. L. et al. Incidence of Hidradenitis Suppurativa and Associated Factors: a Population-Based Study of Olmsted County, Minnesota. J. Invest. Dermatol. 2013;133(1):97–103.
  20. Cosmatos I., Matcho A., Weinstein R. et al. Analysis of Patient Claims Data to Determine the Prevalence of Hidradenitis Suppurativa in the United States. J. Am. Acad. Dermatol. 2013;68(3):412–419.
  21. Onderdijk A. J., van der Zee H. H., Esmann S. et al. Depression in Patients with Hidradenitis Suppurativa. J. Eur. Acad. Dermatol. Venereol. 2013;27(4):473–478.
  22. Matuziak L., Bieniek A., Szipietowski J. C. Psychophysical Aspects of Hidradenitis Suppurativa. Acta Derm. Venereol. 2010;90(3):264–268.
  23. Dufour D. N., Emtestam L., Jemec G. B. Hidradenitis Suppurativa: a Common and Burdensome, Yet Under Recognised Inflammatory Skin Disease. Postgrad. Med. J. 2014;90(1062):216–221.
  24. Margesson L. J., Danby F. W. Hidradenitis Suppurativa. Best Pract. Res. Clin. Obstet. Gynaecol. 2014;28(7):1013–1027.
  25. Zouboulis C. C., Desai N., Emtestam L. et al. European S1 Guideline for the Treatment of Hidradenitis Suppurativa/Acne Inversa. J. Eur. Acad. Dermatol. Venereol. 2015;29(4):619–644.
  26. Kimball A., Jemec G. B., Gu Y. et al. A Novel Hidradenitis Suppurativa Efficacy Variable, HiSCR (Hidradenitis Suppurativa Clinical Response), is Responsive to Change with Adalimumab Therapy: Results of a Phase 2 Study. Paper presented at: 71st Annual Meeting of the American Academy of Dermatology; March 1–5, 2013; Miami Beach, FL.
  27. Wortsman X., Jemec G. B. A 3D Ultrasound Study of Sinus Tract Formation in Hidradenitis Suppurativa. Dermatol. Online J. 2013;19(6):18564.
  28. Jemec G. B., Revuz J. Treatment. In: Hidradenitis Suppurativa. Ed. by G. B. Jemec, J. Revuz, J. L. Leyden. Germany: Springer, 2006. P. 183–186.
  29. Kimball A. B., Jemec G. B., Yang M. et al. Assessing the Validity, Responsiveness and Meaningfulness of the Hidradenitis Suppurativa Clinical Response (HiSCR) as the Clinical Endpoint for Hidradenitis Suppurativa Treatment. Br. J. Dermatol. 2014;171(6):1434–1442.
  30. Shah N. Hidradenitis Suppurativa: a Treatment Challenge. Am. Fam. Physician. 2005;72(8):1547–1552.
  31. van der Zee H. H., Prens E. P., Boer J. Deroofing: a TissueSaving Surgical Technique for the Treatment of Mild to Moderate Hidradenitis Suppurativa Lesions. J. Am. Acad. Dermatol. 2010;63(3):475–480.
  32. Alharbi Z., Kauczok J., Pallua N. A Review of Wide Surgical Excision of Hidradenitis Suppurativa. BMC Dermatol. 2012;12:9.
  33. Gulliver W., Zouboulis C. C., Prens E. 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;17(3):343–351.
  34. Kimball A. B., Jemec G., Armstrong A. et al. Evaluating Optimal Medium-Term Dosing Strategy for Adalimumab in Patients with Moderate-toSevere Hidradenitis Suppurativa Based on Analysis of Integrated Results from the PIONEER I & II Phase 3, Randomized, Placebo-Controlled Trials. Poster presented at: 23rd World Congress of Dermatology (WCD); June 8–13, 2014; Vancouver, Canada. Abstract 3079375.
  35. Kimball A. B., Zouboulis C. C., Armstrong A. W. et al. Safety and Efficacy of Adalimumab in Patients with Moderate to Severe Hidradenitis Suppurativa: Results from First 12 Weeks of PIONEER I, a Phase 3, Randomized, Placebo-Controlled Trial. Paper presented at: 73rd Annual Meeting of the American Academy of Dermatology; March 20–24, 2015; San Francisco, CA.
  36. Jemec G. B., Gottlieb A., Forman S. et al. Efficacy and Safety of Adalimumab in Patients with Moderate to Severe Hidradenitis Suppurativa: Results from PIONEER II, a Phase 3 Randomized Placebo-Controlled Trial. Paper presented at: 73rd Annual Meeting of the American Academy of Dermatology; March 20–24, 2015; San Francisco, CA.
  37. Инструкция по применению препарата Хумира от 12.05.2016. 49 с. [Instructions for the Use of the Drug Humira from 05.12.2016. 49 p.]

Supplementary files

Supplementary Files
Action
1. JATS XML
Download

Copyright (c) 2017 Khobeish M.M., Shustov D.V., Sokolovsky E.V.

Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 International License.
СМИ зарегистрировано Федеральной службой по надзору в сфере связи, информационных технологий и массовых коммуникаций (Роскомнадзор).
Регистрационный номер и дата принятия решения о регистрации СМИ: серия ПИ № ФС 77 - 60448 от 30.12.2014.


This website uses cookies

You consent to our cookies if you continue to use our website.

About Cookies