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Hidradenitis suppurativa

Abstract

Hidradenitis suppurativa (HS; also designated as acne inversa) is a chronic inflammatory disorder, which affects the intertriginous skin and is associated with numerous systemic comorbidities. The estimated prevalence of HS is ~1% in most studied countries. Typically starting in early adulthood, cutaneous inflamed nodules, abscesses and pus-discharging tunnels develop in axillary, inguinal, gluteal and perianal body sites. The comorbidities of HS include metabolic and cardiovascular disorders, which contribute to reduced life expectancy. A genetic predisposition, smoking, obesity and hormonal factors are established aetiological factors for HS. Cutaneous changes seem to start around hair follicles and involve activation of cells of the innate and adaptive immune systems, with pivotal roles for pro-inflammatory cytokines such as tumour necrosis factor, IL-1β and IL-17. The unrestricted and chronic immune response eventually leads to severe pain, pus discharge, irreversible tissue destruction and scar development. HS has profound negative effects on patients’ quality of life, which often culminate in social withdrawal, unemployment, depression and suicidal thoughts. The therapeutic options for HS comprise antibiotic treatment, neutralization of tumour necrosis factor and surgical intervention together with lifestyle modification. Nevertheless, there is an enormous need for awareness of HS, understanding of its pathogenesis and novel treatments.

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Fig. 1: Histology of hidradenitis suppurativa lesions.
Fig. 2: Pathogenesis of HS — initial pathogenetic events.
Fig. 3: Pathogenesis of HS — progression to advanced disease.
Fig. 4: Typical skin lesions of hidradenitis suppurativa.
Fig. 5: Hidradenitis suppurativa management.

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Acknowledgements

The authors thank A. Vossen (Erasmus University Medical Center), G. Kokolakis (Charité – Universitätsmedizin Berlin), I. Chlebicka (Wrocław Medical University), K. van Straalen (Erasmus University Medical Center) and J. Triebus (Charité – Universitätsmedizin Berlin) for their help with this manuscript. The authors also acknowledge the support by the German Federal Ministry of Education and Research (http://www.bmbf.de/; grant 01ZX1312A to K.W. and R.S.).

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Authors

Contributions

Introduction (R.S.); Epidemiology (G.B.E.J.); Mechanisms/pathophysiology (K.W.); Diagnosis, screening and prevention (E.P.); Management (G.B.E.J.); Quality of life (Ł.M.); Outlook (A.B.K. and R.S.); Overview of Primer (R.S.). R.S. and G.B.E.J. contributed equally and are co-first authors. E.P. and K.W. contributed equally and are co-last authors.

Corresponding author

Correspondence to Robert Sabat.

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Competing interests

R.S. is a member of Arbeitsgemeinschaft Dermatologische Forschung (ADF; Consortium for Dermatological Research), the German Society of Allergy and Clinical Immunology (DGAKI), the German Society of Immunology (DGfI), the American Association of Immunologists (AAI), the International Psoriasis Council (IPC) and the European Hidradenitis Suppurativa Foundation (EHSF), and is the spokesman of the ADF Psoriasis Group. R.S. has received research grants and scientific awards or honoraria for participation on advisory boards, clinical trials or as speaker for one or more of the following: AbbVie Inc., AbbVie Deutschland GmbH & Co. KG, Bayer Schering Pharma AG, Biogen IDEC GmbH, Boehringer Ingelheim Pharma GmbH & Co. KG, Celgene GmbH, Celgene International II Sàrl, Charité Research Organization GmbH, Dr. Willmar Schwabe GmbH & Co. KG, Flexopharm GmbH & Co. KG, Generon Corporation Ltd., Janssen-Cilag GmbH, La Roche-Posay Laboratoire Dermatologique Deutschland, Novartis Pharma GmbH, Parexel International GmbH, Pfizer Deutschland GmbH, Sanofi-Aventis Deutschland GmbH, TFS Trial Form Support GmbH, UCB Pharma GmbH. G.B.E.J. is the vice president and a founding member of the EHSF, a board member of the Nordic Association of Dermatology and an honorary member of the British Association of Dermatologists. G.B.E.J. is also a member of the European Academy of Dermatology and Venereology, the American Academy of Dermatology, the Serbian Association of Dermatovenereologists, the Hungarian Society of Dermatology, the Baltic Association of Dermatovenerologists, the Finnish Society of Dermatology and the Latvian Society of Dermatology and Venereology. G.B.E.J. is the Editor-in-chief of Dermatology, commissioning editor of Clinical Problems in Dermatology, associate editor of Clinical Dermatology and Acta Dermatovenerologica Adriatica, Pannonica et Alpina, and an editorial board member of Acta Dermatovenerologica Croatica and Frontiers in Medicine. G.B.E.J. has received research grants and grants for participation as an investigator from Abbvie, Astra-Zeneca, Inflarx, Janssen-Cilag, Leo Pharma, Novartis, Regeneron and Sanofi. He has also received unrestricted departmental grants from Abbvie, Leo Pharma, and Novartis. G.B.E.J. has received honoraria from AbbVie, Chemocentryx, Coloplast, Incyte, Inflarx, Novartis, Pierre Fabre and UCB for participation on advisory boards, and has received speaker honoraria from AbbVie, Boehringer-Ingelheim, Galderma, MSD and Novartis. Ł.M. is a founding member of the EHSF and a member of the European Academy of Dermatology and Venereology and Polish Dermatological Society. Ł.M. has received research grants, travel grants, consulting honoraria or lecturer’s honoraria from AbbVie, Amgen, Behringer Ingelheim, InfraRX, Janssen, Leo Pharma, Medac, Menlo Therapeutics, Pfizer, Pierre Fabre, Polpharma, Regeneron, Novartis, Trevi, UCB and Valeant. A.B.K. is a consultant and investigator for Novartis, Abbvie, UCB, Pfizer, Lilly and Janssen. A.B.K. has received fellowship funding from Janssen and Abbvie. K.W. has received research grants, travel grants, consulting honoraria or lecturer’s honoraria from AbbVie, Bayer Schering Pharma, Biogen IDEC, Celgene, Dr. Willmar Schwabe GmbH & Co. KG, Flexopharm, Generon Corporation, Janssen-Cilag, Johnson & Johnson, Novartis, Pfizer, Sanofi-Aventis, TFS Trial Form Support and UCB. E.P. declares no competing interests.

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Glossary

Nodules

Firm swellings of the skin, mainly arising from the dermis and subcutis.

Abscesses

Red, tender, pus-containing cavities in the skin or any organ, surrounded by inflammation.

Sinus tracts

Linear, palpable, subcutaneous tunnels draining to the skin surface via an opening.

Fistulas

Permanent, abnormal tunnels between two hollow organs or from a hollow organ to the skin surface.

Plugging

The occlusion of a hair follicle or sweat gland pore by a keratin mass.

Open comedones

Wide skin pores, plugged with a mass of black keratin debris.

Double-ended pseudocomedones

Two interconnected dilated pores with a plug of black keratin debris at each end.

Pilonidal sinus

A recurrent nodule or abscess draining via a sinus in the cleft between the buttocks near the tailbone.

Furuncle

An abscess or nodule arising from a hair follicle, caused by infection with Staphylococcus aureus.

Pyoderma gangrenosum

Rare skin disease characterized by growing painful ulcers with undermined borders.

Acne conglobata

A rare and severe form of acne presenting with interconnected nodules, abscesses and sometimes sinus tracts, mostly in the neck, back and chest.

Dissecting cellulitis of the scalp

A rare condition of the scalp associated with hidradenitis suppurativa, with pus-filled or indurated lumps, scarring and permanent hair loss.

Pyogenic arthritis

Arthritis caused by invasion of a joint by an infectious agent resulting in painful joint inflammation.

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Sabat, R., Jemec, G.B.E., Matusiak, Ł. et al. Hidradenitis suppurativa. Nat Rev Dis Primers 6, 18 (2020). https://doi.org/10.1038/s41572-020-0149-1

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