Hidradenitis suppurativa

Hidradenitis suppurativa ( HS ) is a chronic , inflammatory , recurrent debilitating disease of the 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 axilla ,
 inguinal and anogenital regions
 Also known as Verneuil’s disease or acne inversa

Epidemiology- Prevalence appears to be 1 % to 4 % of the general population Occurs more commonly in women ( reports controversial ) Females more likely to have a family history and men had a tendency for more severe disease and associated acne Disease of the young- average age at presentation is 26 yrs for ♀ and 31 for ♂ No racial predilection

Cause- Cause unclear – complex disease
 Multifactorial and various factors can play a role as
○ occlusion of the apocrine duct by a keratinous plug
○ defects of follicular epithelium and bacterial overgrowth
○ role of hormones- happens after puberty and can flare with menstrual cycle
○ smoking – role of nicotine
○ autoimmune – associated with certain disorders as thyroid disease , 
Inflammatory bowel disease, Sjogren’s syndrome
 Acne vulgaris
 Obesity Athletes Genetic component Host defense defects Chemical irritants ( eg deodorants ) and mechanical irritation ( depilation , shaving ) Heat ( ↑ in summer ) Oral contraceptive pill Zinc deficiency – may predispose

Disease burden- Chronic disabling disorder Relentless progression and can cause keloids , contractures & immobility Disease can progress to scarring and sinus formation Distressing , recurrent disease that impairs quality of life – sig psychological impact Risk of depression , sexual impairment Often misdiagnosed and patients undergo repeat and unnecessary investigations

Hurley staging- Stage 1 – abscess formation single or multiple without sinus tracts or scarring Stage 2 – recurrent abscesses with tract formation and cicatrisation , single or multiple , widely separated lesions Stage 3 – diffuse or near-diffuse involvement or multiple interconnected tracts and abscesses across the entire area

Disease classification – consider using 
Hurley staging – treatment will depend on disease severity.Often poor response to previous antibiotic therapy

Pyoderma Arthritis Inflammatory bowel disease- eg Crohn’s Sponyloarthropathy Anaemia Metabolic syndrome

Pain management Weight loss Smoking cessation Treatment of superinfection Dressings if suitable Warm compresses Screen for depression

Hidradenitis complications – Contractures and reduced mobility Sinus tracts , fistula , scarring Squamous cell carcinoma Lymphoedema Localised infection / cellulitis Chronic pain Systemic amyloidosis Possibly anaemia

Consider referring early as treatment often multidisciplinary – aim is to
 prevent or limit scarring. A recent high-quality cross sectional study of more than 40,000 patients and meta analysis of prior studies suggests a 1.5 to 3 fold risk of type 2 diabetes in patients with HS , with a prevalence of up to 30 %. Patients with physical signs of diabetes , hypertension, obesity , and / or hyperlipidaemia are at a higher risk and should be screened. 
Risk of PCOS is also increased significantly.

Clindamycin topical ( first line ) Metronidazole topical Chlorhexidine topical Hexachlorophene topical Dermol 500 lotion wash Tetracycline 500 mg bd for 2 months
PCDS suggests Lymecycline 408 mg od Doxycyline 100 mg bd for 2 months Minocycline 100 mg bd for 2 months Combination therapy
Clindamycin 300 mg bd + Rifampicin 300 mg bd for 3 months ( PCDS ) Flucloxacillin can be used as a short course for acute flare ups ( BAD )

OTHER TREATMENT OPTIONS AS PER BAD 
GUIDELINES
 Combination treatment if no response to initial treatment with oral tetracycline Acitretin ( specialist use ) Dapsone ( specialist use ) Metformin consider for those who have diabetes and HS or in females with HS and PCOS/ pregnancy

LINKS AND RESOURCES

PATIENT INFORMATION

BAD leaflet on HS http://www.bad.org.uk/shared/get-file.ashx?id=88&itemtype=document

Dermnet NZ on HS –https://dermnetnz.org/topics/hidradenitis-suppurativa/

HS Foundation with resources for patients and professionals https://www.hs-foundation.org/

HS Trust for patients in UK https://www.hstrust.org/

British Skin Foundation on HS https://www.britishskinfoundation.org.uk/hidradenitis-suppurativa

FURTHER READING FOR CLINICIANS

Canadian Family Physician on HS https://www.cfp.ca/content/cfp/63/2/114.full.pdf

BAD guideline on management of HS https://www.ncbi.nlm.nih.gov/pubmed/30552762

A comparison of international management guidelines on HS https://www.karger.com/Article/FullText/503605

North American clinical management guidelines for hidradenitis suppurativa: A publication from the United States and Canadian Hidradenitis Suppurativa Foundations https://www.jaad.org/article/S0190-9622(19)30367-6/fulltext

European S1 guideline on management of HS https://www.epgonline.org/uk/guidelines/european-s1-guideline-for-the-treatment-of-hidradenitis-suppurativa-acne-inversa.html

References

1. Diagnosis and management of hidradenitis suppurativa BMJ 2013 ; 346 ;f2121
2. Medscape Hidradenitis Suppurativa Aug 2017 Marina Jovanovic et al https://emedicine.medscape.com/article/1073117-overview
3. Ingram, John R et al. “Interventions for hidradenitis suppurativa.” The Cochrane database of systematic reviews vol. 2015,10 CD010081. 7 Oct. 2015, doi:10.1002/14651858.CD010081.pub2
4. Ingram JR. Interventions for Hidradenitis Suppurativa: Updated Summary of an Original Cochrane Review. JAMA Dermatol. 2017;153(5):458–459. doi:10.1001/jamadermatol.2017.0432)
5. BMJ Best Practice Hidrandeitis suppurativa https://bestpractice.bmj.com/topics/en-gb/1047
6. Zouboulis, C., Desai, N., Emtestam, L., Hunger, R., Ioannides, D., Juhász, I., Lapins, J., Matusiak, L., Prens, E., Revuz, J., Schneider‐Burrus, S., Szepietowski, J., van der Zee, H. and Jemec, G. (2015), European S1 guideline for the treatment of hidradenitis suppurativa/acne inversa. J Eur Acad Dermatol Venereol, 29: 619-644. doi:10.1111/jdv.12966
7. RACGP Hidradenitis suppurativa – Management , comorbidities and monitoring https://www.racgp.org.au/afp/2017/august/hidradenitis-suppurativa-management-comorbidities-and-monitoring/
8. Gulliver, Wayne et al. “Evidence-based approach to the treatment of hidradenitis suppurativa/acne inversa, based on the European guidelines for hidradenitis suppurativa.” Reviews in endocrine & metabolic disorders vol. 17,3 (2016): 343-351. doi:10.1007/s11154-016-9328-5
9. Hidradenitis Suppurativa : A Treatment Challenge

   NIPA SHAH, M.D., University of Illinois College of Medicine, Chicago, Illinois Am Fam Physician. 2005 Oct 15;72(8):1547-1552.

10. First Consult Hidradenitis Suppurativa April 2011
11. Hidradenitis suppurativa : Treatment of Skin Disease : Comprehensive Therapeutic Strategies , 101 , 314-315
12. Yuan JT, Naik HB. Complications of hidradenitis suppurativa. Seminars in Cutaneous Medicine and Surgery. 2017 Jun;36(2):79-85. DOI: 10.12788/j.sder.2017.022.
13. Hidradenitis suppurativa (syn. acne inversa) Primary Care Dermatology Society via http://www.pcds.org.uk/clinical-guidance/hidradenitis-suppurativa#management
14. Ingram, J., Collier, F., Brown, D., Burton, T., Burton, J., Chin, M., Desai, N., Goodacre, T., Piguet, V., Pink, A., Exton, L. and Mohd Mustapa, M. (2019), British Association of Dermatologists guidelines for the management of hidradenitis suppurativa (acne inversa) 2018. Br J Dermatol, 180: 1009-1017. doi:10.1111/bjd.17537
15. North American clinical management guidelines for hidradenitis suppurativa: A publication from the United States and Canadian Hidradenitis Suppurativa Foundations Alikhan, Ali et al. Journal of the American Academy of Dermatology, Volume 81, Issue 1, 91 – 101 https://plu.mx/plum/a/?doi=10.1016/j.jaad.2019.02.068
16. Schmitt, Juliano Vilaverde et al. “Risk factors for hidradenitis suppurativa: a pilot study.” Anais brasileiros de dermatologia vol. 87,6 (2012): 936-8. doi:10.1590/s0365-05962012000600024