Dear colleagues

Hidradenitis suppurativa/acne inversa is a chronic, inflammatory, recurrent, debilitating skin disease (of the terminal hair follicle) that usually presents after puberty with painful, deep-seated, inflamed lesions in the apocrine gland-bearing areas of the body, most commonly the axillary, inguinal, and anogenital regions (Dessauer definition).¹

¹ Kurzen H, Kurokawa I, Jemec GBE, Emtestam L, Sellheyer K, Giamarellos-Bourboulis EJ, Nagy I, Bechara FG, Sartorius K, Lapins J, Krahl D, Altmeyer P, Revuz J, Zouboulis CC. What causes hidradenitis suppurativa? Exp Dermatol 17:455–472, 2008

Diagnostic procedure

You can find information on the diagnostic procedure and criteria of hidradenitis suppuratina under:

  • Zouboulis CC, del Marmol V, Mrowietz U, Prens E, Tzellos T, Jemec GBE. Hidradenitis suppurativa/acne inversa: Criteria for diagnosis, severity assessment, classification and disease evaluation. Dermatology 231:184-190, 2015

Photographic documentation

A guide for photographic documentation can be seen in:

  • Zouboulis CC, Nogueira da Costa A. Standardized photographic documentation of hidradenitis suppurativa/acne inversa. Dermatology 235:51-54, 2019

Guidelines for treatment

Guidelines for treatment are published under:

  • Zouboulis CC, Desai N, Emtestam L, Hunger RE, Ioannides D, Juhász I, Lapins J, Matusiak L, Prens EP, Revuz J, Schneider-Burrus S, Szepietowski JC, van der Zee HH, Jemec GBE. European S1 guideline for the treatment of hidradenitis suppurativa / acne inversa. J Eur Acad Dermatol Venereol 29:619-644, 2015
  • Gulliver W, Zouboulis CC, Prens E, Jemec GB, Tzellos T. Evidence-based approach to the treatment of hidradenitis suppurativa/acne inversa, based on the European guidelines for hidradenitis suppurativa. Rev Endocr Metab Disord 17:443-451, 2016
  • Zouboulis CC, Bechara FG, Dickinson-Blok JL, Gulliver W, Horváth B, Hughes R, Kimball AB, Kirby B, Martorell A, Podda M, Prens EP, Ring HC, Tzellos T, van der Zee HH, van Straalen KR, Vossen ARJV, Jemec GBE. 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 33:19-31, 2019

Severity classification system

A severity classification system and outcome measure for treatment effectiveness is published under:

  • Zouboulis CC, Tzellos T, Kyrgidis A, Jemec GBE, Bechara FG, Giamarellos-Bourboulis EJ, Ingram JR, Kanni T, Karagiannidis I, Martorell A, Matusiak L, Pinter A, Prens EP, Presser D, Schneider-Burrus S, von Stebut E, Szepietowski JC, van der Zee HH, Wilden SM, Sabat R, on behalf of the EHSF Investigator Group. Development and validation of IHS4, a novel dynamic scoring system to assess hidradenitis suppurativa/acne inversa severity. Br J Dermatol 177:1401-1409, 2017

Information on comorbid disorders

Information on comorbid disorders can be found in:

  • Fimmel S, Zouboulis CC. Comorbidities of hidradenitis suppurativa (acne inversa). Dermatoendocrinol 2:9-16, 2010
  • Tzellos T, Zouboulis CC, Gulliver W, Cohen AD, Wolkenstein P, Jemec GB. Cardiovascular disease risk factors in patients with hidradenitis suppurativa: a systematic review and meta-analysis of observational studies. Br J Dermatol 173:1142-1155, 2015
  • Tzellos T, Zouboulis CC. Review of comorbidities of hidradenitis suppurativa: Implications for daily clinical practice. Dermatol Ther (Heidelb) 10:63-71, 2020

Concepts on disease etiology

Theoretical and experimental concepts on disease etiology can be found under:

  • Tricarico P, Boniotto M, Genovese G, Zouboulis CC, Marzano AV, Crovella S. Skin-OMICS: an integrated approach to unravel hidradenitis suppurativa etiopathogenesis. Front Immunol 10:892, 2019
  • Jenei A, Dajnoki Z, Medgyesi B, Gáspár K, Béke G, Kinyó Á, Méhes G, Hendrik Z, Dinya T, Törőcsik D, Zouboulis CC, Prens EP, Bíró T, Szegedi A, Kapitány A. Apocrine gland-rich skin has a non-inflammatory IL-17 related immune milieu, which turns to inflammatory IL-17 mediated disease in hidradenitis suppurativa. J Invest Dermatol 139:964-968, 2019
  • Zouboulis CC, Nogueira da Costa A, Makrantonaki E, Hou XX, Almansouri D, Dudley JT, Edwards H, Readhead B, Balthasar O, Jemec GBE, Bonitsis NG, Nikolakis G, Trebing D, Zouboulis KC, Hossini AM. Alterations in innate immunity and epithelial cell differentiation are the molecular pillars of hidradenitis suppurativa. J Eur Acad Dermatol Venereol [Epub ahead of print], 2019

Information on quality of life:

Information on quality of life can be found under:

  • Chernyshov PV, Zouboulis CC, Tomas-Aragones L, Jemec G, Manolache L, Tzellos T, Sampogna F, Evers AWM, Dessinioti C, Marron SE, Bettoli V, van Cranenburgh O, Svensson A, Liakou A, Poot F, Szepietowski JC, Salek MS, Finlay AY. Quality of life measurement in acne. Position paper of the European Academy of Dermatology and Venereology Task Forces on Quality of Life and Patient Oriented Outcomes and Acne, Rosacea and Hidradenitis Suppurativa. J Eur Acad Dermatol Venereol 32:194-208, 2018
  • Chernyshov PV, Zouboulis CC, Tomas-Aragones L, Jemec GB, Svensson A, Manolache L, Tzellos T, Sampogna F, Pustisek N, van der Zee HH, Marron SE, Spillekom-van Koulil S, Bewley A, Linder D, Abeni D, Szepietowski JC, Augustin M, Finlay AY. Quality of life measurement in hidradenitis suppurativa: position statement of the European Academy of Dermatology and Venereology task forces on Quality of Life and Patient-Oriented Outcomes and Acne, Rosacea and Hidradenitis Suppurativa. J Eur Acad Dermatol Venereol 33:1633-1643, 2019

CONSENSUS FOR ANTI- COVID-19 MEASUREMENTS IN HS PATIENTS

Giamarellos-Bourboulis EJ, Bettoli V, Jemec GBE, et al. Anti-COVID-19 measurements for hidradenitis suppurativa patients. Exp Dermatol 2021;30(suppl 1):18–22

Taking into consideration the available data, the experts of the symposium “Anti-COVID-19 measurements in HS patients”, which took place during the 10th International Conference of the EHSF e.V. reached unanimously a consensus on behalf of the European Hidradenitis Suppurativa Foundation e.V., which includes the following measures:

  • The prevalence of COVID-19 in patients with HS is apparently lower than that of the general population. This may be due to HS patients having been especially careful with social shielding. However, underestimation is also possible.
  • HS appears not to be associated with a more severe course of COVID-19.
  • Treatment of HS with antibiotics and the TNF-α inhibitor adalimumab seems not to increase the risk for COVID-19 or induce a more severe course.
  • Treatment initiation of HS patients with other therapeutic compounds should be carefully evaluated at individual level.
  • Even if we do not yet know what effect—if any—vaccination has on HS (and vice versa), anti-SARS-CoV2 vaccination is recommended. Benefits versus risks should be considered at an individual level.
  • Treatment with adalimumab appears to be compatible with anti-SARS-CoV2 vaccination and should not be interrupted, especially in moderate-to-severe HS. It might be suspended around the time point of anti-SARS-CoV2 vaccination.
  • It is still unclear if biologics may compromise SARS-CoV2 vaccination. Serological confirmation of successful vaccination by ELISA is still not recommended as routine practice.
  • National self-protection and hygiene measurements should be adhered to. Self-protection with masks is recommended even despite vaccination.
  • This consensus is based on expert opinion and literature evidence, and complements the recommendations already published by other scientific societies.

Based on the available knowledge, HS patients may be vaccinated and the group of them, affected with metabolic syndrome, at the soonest possible time. HS patients on adalimumab treatment can be vaccinated with non-living virus anti-SARS-CoV2 vaccines. A possible suboptimal effect of the vaccine may be suspected. The management of the biological treatment in HS patients is at the discretion of the dermatologist / responsible physician.

Systemic Therapy for Patients with Hidradenitis suppurativa/Acne inversa during the pandemic phase of SARS-CoV-2 (Coronavirus)

Dear colleagues,

the ongoing coronavirus (COVID-19) pandemic is a serious public health concern. It  is not known to what extent the coronavirus impacts Hidradenitis suppurativa/Acne inversa and its treatment. The European Hidradenitis Supurativa Foundation (EHSF) e.V. advises physicians to be alert to the potentially harmful effects of COVID-19 infection on patients with Hidradenitis suppurativa/Acne inversa and counsel all their patients on how to prevent transmission of the virus.

It is recommended that patients with symptoms, initially consult their doctor by email, fax or telephone. For Hidradenitis suppurativa/Acne inversa patients diagnosed with COVID-19 disease, the EHSF recommends physicians to discontinue the use of immunosuppressant medications. This is in accordance with established psoriasis treatment guidelines for psoriasis (for example, the European Dermatology Forum and the American Academy of Dermatology), which recommend the discontinuation of immunosuppressive treatments in patients with active infections.

As of now, there is insufficient evidence to determine how COVID-19 will impact Hidradenitis suppurativa/Acne inversa patients on systemic treatment. Patients using TNF-alpha blockers (adalimumab, infliximab, etanercept) may have a slightly increased risk of upper repiratory tract infections, especially for infliximab. Particular caution is, therefore, recommended for individuals generally at risk of developing a more serious course of COVID-19 disease: Men, age 60+ years and patients with cardiovascular disease, pulmonary diseases or diabetes.

In the event of acute fever or respiratory symptoms and other clinical suspicion of a COVID-19 infection,  it is advised that a diagnosis is carried out following the current recommendations of the respective authorities. If medication is imminent, it is recommended that it is postponed until a diagnosis is made.The EHSF will continue to provide updates to the global dermatology community.

For the latest information on the coronavirus (COVID-19) outbreak, including prevention and signs and symptoms of the disease, please refer to the World Health Organization (WHO) website.

Prof. Dr. Veronique del Marmol, Brussels, Belgium
Prof. Dr. Gregor B.E. Jemec, Roskilde, Denmark
Prof. Dr. Christos C. Zouboulis, Dessau, Germany
Prof. Dr. Errol Prens, Rotterdam, The Netherlands
Prof. Dr. Thrasyvoulos Tzellos, Harstad, Norway