Introduction to Hidradenitis Suppurativa
Authors: Miroslava Verbat, med. student, Ema Šutáková, med. student, Dominika Miklišová med. student, Julia Bartkova, MD
Hidradenitis suppurativa, also called Verneuil’s disease or acne inversa is a chronic inflammatory skin condition characterized by lesions located in hair follicles (1).
It is described as a pyoderma of the adnexal structures, more specifically of the apocrine glands, located on the scalp, neck, axilla, breast, periumbilical, inguinal, perianal, and gluteal regions, creating fistulas, abscesses, and fibrosis.
The disease is not common, within Europe the prevalence is 1%.
Additionally, it is often misdiagnosed for folliculitis, acne vulgaris or cutaneous Crohn disease therefore delaying the accurate diagnosis up to years (2).
(5) Hidradenitis suppurativa can be classified according to the severity of the lesions observed:
- Hurley Stage I (Mild): single or multiple isolated inflammatory nodule and abscesses formation, without sinus tracts and scarring.
- Hurley Stage II (Moderate): recurrent abscesses with >1 sinus tracts and scarring, separated by intact skin. We can observe solitary or multiple lesions.
- Hurley Stage III (Severe): multiple lesions with interconnected sinus tracts, coalescing into inflammatory plaques with severe scarring, and abscesses across the entire region.
The exact etiology of hidradenitis is not known, although we can describe multiple predisposing factors:
- Genetics, 1/3 of the patients have a positive family history
- Female sex
- Dysregulation of inflammatory pathways,
- Bacterial colonization (Staphylococcus Aureus infection)
- Drugs (lithium therapy, contraception with higher to progesterone to estrogen ratio and levonorgestrel intrauterine device)
- Others: seronegative arthritis, spondylarthritis, rheumatoid arthritis, anemia, psoriasis and systemic lupus erythematosus.
Moreover, clinical observations in female patients have confirmed that hormonal changes like the menstrual cycle, endocrine disorders such as polycystic ovary syndrome PCOS, metabolic syndrome, influence flare ups (6). This plays an additional role for its management.
- Topical lincosamide applied twice daily for 3 months on local lesions (abscesses, nodules and pustules) is the first line treatment for Hurley stage I.
- Resorchinol, a topical agent with keratolytic, antipruritic and anti-inflammatory qualities can be applied twice daily on active inflammatory lesions.
- Intralesional injections with corticosteroids can also be considered on solitary lesions.
- Tetracyclines (for 10 weeks) is the treatment of choice for more broadly spread Hurley stage I and II. Clindamycin and rifampin (for 10 weeks) can be preferred for more severe Hurley stage II which is unresponsive to tetracyclines.
- Carbapenems can give rapid improvement (although it is given often as neoadjuvant therapy for bridging surgery (6).
Due to the strong link between endocrine imbalances and hidradenitis suppuritiva, anti-androgenic therapy can be introduced (for example, hinylestradiol and cyproteronacetate (6).
- Retinoids such as acitretin can be used for a special subtype of hidradenitis with follicular phenotype.
- Adalimumab (a IgG1 monoclonal antibody specific for TNF alpha, sold under the name HUMIRA) 40 mg every 3 weeks, subcutaneous injections is recommended for high stages disease with severe, extensive sinus tract, fistula formation and sclerosis (7).
Most patients require adjuvant medical therapies (topical or systemic) to diminish the inflammation and surgical treatment (drainage of tense abscesses in acute scenarios for immediate pain relief) (3).
The British Association of Dermatologists guidelines for the management of HS recommend surgical treatment for recurrent and severe cases which are not responsive to oral antibiotics, acitretin, or adalimumab (4).
We can divide the surgical management of hidradenitis into 3 types:
- Incision and drainage – for rapid pain relief. It presents with a 100 % recurrence rate,
- Deroofing – preserving the floor allows for the epithelial cells from sweat glands and hair follicles remnants to quickly reepithelize and heal by secondary intention – only 27% of recurrence was observed,
- Excision – more invasive option aimed to remove the affected tissue in its integrity. We can further subdivide it into 1) Limited/local excision, 2) Wide excision, 3) Radical excision. The recurrence rate is between 22 and 13%
Various options are possible for closure of the wound:
- Grafts, STSG (split thickness and full thickness skin grafts),
- Flaps (if vascular channels and nerves are exposed) – lateral thoracic flap, fasciocutaneous V-Y flap, Limberg flap, musculocutaneous flap and thoracodorsal artery perforator flap,
- Healing by secondary intention (the wound will be left to heal by itself).
The treatment option can vary according to the stage of the disease.
Even though extensive surgery is often considered as the best option to minimize recurrence, less invasive interventions using a neodymium-doped yttrium aluminium garnet laser (Nd:YAG) can be recommended for Hurley stage II and III disease, additionally CO2 laser or surgical deroofing can also be used if the lesions present fibrotic scarring and nodules.
As a preventive measure depilation can also be suggested (3).
Overall, hidradenitis is difficult to diagnose and often refractory to conventional treatment which make its treatment and management very challenging for clinicians. A multidisciplinary approach is necessary.
1. M.A. García-Sánche. Verneuil’s disease: A case report and literature review. Mexican Journal of Gastroenterology. Published July 2015. Accessed April 2023. http://www.revistagastroenterologiamexico.org/en-verneuil39s-disease-a-case-report-articulo-S2255534X15000377
2. Georgios Kokolakis. Delayed Diagnosis of Hidradenitis Suppurativa and Its Effect on Patients and Healthcare System. Karger Journal. Published July 1 2020. Accessed May 2023. https://pubmed.ncbi.nlm.nih.gov/32610312/
3. Ratnakar Shukla. Surgical Treatment in Hidradenitis Suppurativa. Journal of Clinical Medicine. Published May 2022. Accessed April 2023. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9101712/
4. J.R. Ingram. British Association of Dermatologists guidelines for the management of hidradenitis suppurativa (acne inversa). British Journal of Dermatology. Published in 2018. Accessed April 2023. https://bdng.org.uk/wp-content/uploads/2022/04/BAD-Guidelines.pdf
5. Victoria Amat- Samaranch. New perspectives on the treatment of hidradenitis suppurativa. Sage journals. Published November 23, 2021. Accessed April 2023. https://journals.sagepub.com/doi/full/10.1177/20406223211055920
6. Lennart Ocker. Current Medical and Surgical Treatment of Hidradenitis Suppurativa / A Comprehensive Review. Journal of Clinical Medicine. Published 6 December 2022. Accessed April 2023. https://mdpi-res.com/d_attachment/jcm/jcm-11-07240/article_deploy/jcm-11-07240.pdf?version=1670329917
7. Ricardo Blanco, MD. Long-term Successful Adalimumab Therapy in Severe Hidradenitis Suppurativa. Jama Dermatology. Published May 2009. Accessed April 2023. https://jamanetwork.com/journals/jamadermatology/fullarticle/712030