What is hidradenitis suppurativa?
Hidradenitis suppurativa is an inflammatory skin disease that is characterised by recurrent boil-like lumps (nodules and abscesses) that culminate in pus-like discharge, difficult-to-heal open wounds (sinueses) and scarring. It commonly occurs on apocrine sweat gland-bearing skin such as in the groin, the underarms (axillae) and under the breasts.
The term "hidradenitis" implies it starts as an inflammatory disorder of sweat glands, which is now known to be incorrect. Hidradenitis suppurativa is also known as ‘acne inversa’ (inverse acne).
What causes hidradenitis suppurativa and who gets it?
The exact cause of hidradenitis suppurativa remains unclear. The condition is a disorder of follicular occlusion. This begins with follicular plugging that obstructs the apocrine gland ducts and leads to perifolliculitis (inflammation around the ducts). This is followed by rupture of the follicular epithelium, bacterial infection and formation of sinus tracts between abscesses under the skin, ie the characteristic symptoms and signs of hidradenitis suppurativa.
The following are thought to play a role in the development of hidradenitis suppurativa.
- Genetics – hidradenitis suppurativa may affect multiple members of a family
- Sex hormones – apocrine sweat glands are stimulated by androgen and suppressed by oestrogen (the exact role of these hormones remains controversial). Progesterone-only contraceptives or devices have been reported to aggravate hidradenitsi suppurativa.
- Endocrine factors – obesity, polycystic ovaries, hirsutism, acne are common findings among women with hidradenitis suppurativa
- Pro-inflammatory cytokines (messenger proteins) that drive inflammation
- Cigarette smoking – hidradenitis suppurativa occurs more frequently in smokers than non-smokers
- Secondary bacterial infection
Women are affected by hidradenitis suppurativa three times as often as men; the reason for this is unknown. The condition most commonly occurs between 20–40 years and coincides with the post-pubertal increase in androgen levels. Disease onset rarely occurs before puberty and after menopause.
In some people, hidradenitis suppurativa makes up one part of follicular occlusion syndrome, when it is associated with acne conglobata, dissecting cellulitis and pilonidal sinus. Hidradenitis suppurativa is also associated with Crohn disease.
Rare genetic autoinflammatory syndromes have been described in which hidradenitis suppurativa is a prominent features. Abnormalities of the PSTPIP1 gene on Chromosome 15q24-q25.1 are described (see PAPA syndrome).
- PASH syndrome: pyoderma gangrenosum, acne and hidradenitis suppurativa
- PAPASH syndrome: Pyogenic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa
What are the signs and symptoms of hidradenitis suppurativa?
The extent and severity of hidradenitis suppurativa varies widely between individuals. Initially, a firm pea-sized papule or nodule (0.5–1.5 cm diameter) may appear on one site, eg the armpit or groin. It may resemble an acne pimple. These lesions may resolve spontaneously, or, within hours to days, they may rupture and ooze a pus-like discharge. The nodules may heal without treatment. At a later time new lesions recur in the adjacent area and may include larger nodules, pseudocysts and abscesses, sinus tract formation, and involvement of multiple sites.
Hidradenitis suppurativa may spread to involve both armpits, on and under the breasts, the nape of the neck, waistband and inner thighs. Anogenital involvement most commonly affects the groin, mons pubis, vulva (in females), sides of the scrotum (in males), perineum, buttocks and perianal folds. The abscesses and sinus tracts can be painful.
How is severity and extent of hidradenitis assessed?
Severity and extent of a disease should be recorded at assessment and when determining the impact of a treatment. Photography of affected areas can also aid follow-up. Two scoring systems are in common use: Hurley Stages and Sartorius Hidradenitis Suppurativa Score.
The Hurley system describes three distinct clinical stages.
- Stage 1 – solitary or multiple, isolated abscess formation without scarring or sinus tracts
- Stage 2 – recurrent abscesses, single or multiple widely separated lesions, with sinus tract formation
- Stage 3 – diffuse or broad involvement, with multiple interconnected sinus tracts and abscesses.
Sartorius Hidradenitis Suppurativa Score
The Sartorius Hidradenitis Suppurativa Score is made by counting involved regions, nodules and sinus tracts.
- Anatomic region involved (axilla, groin, genital, gluteal, or other inflammatory region left and/or right): 3 points per region involved
- Number and scores of lesions (abscesses, nodules, fistulas, scars): 2 points for each nodule, 4 points for each fistula, 1 point for each scar, 1 point each for "other"
- Longest distance between 2 relevant lesions (ie, nodules and fistulas, in each region, or size if only 1 lesion): Less than 5 cm, 2 points; less than 10 cm, 4 points; more than 10 cm, 8 points
- Lesions clearly separated by normal skin in each region: If yes, 0 points; if no, 6 points
The six-point Physician Global Assessment (PGA) ranges from clear to very severe. It is used in clinical trials to measure clinical improvement in inflammatory nodules, abscesses and draining fistulae.
The Hidradenitis Suppurativa Clinical Response (HiSCR) is defined as a ≥ 50% reduction in inflammatory lesion count (abscesses + inflammatory nodules), and no increase in abscesses or draining fistulas when compared with baseline. It has been recently used to assess the effectiveness of treatment with biologics.
It is also useful to consider the degree of pain, the number of flares, and the impact on daily life in hidradenitis suppurativa. The Cardiff Dermatology Life Quality Index or DLQI questionnaire is often used.
What factors are associated with severe disease?
Severe hidradenitis (Hurley Stage 3) has been associated with:
- Male gender
- Axillary and perianal involvement
- Disease duration
What is the treatment for hidradenitis suppurativa?
Medical management of hidradenitis suppurativa is difficult. The aim is to catch the disease in its early stages and treat and control these milder forms. Antibiotics are usual therapy (partly to eradicate secondary bacterial infection and partly for their anti-inflammatory action) and long courses may be necessary. Weight loss in obese patients and smoking cessation are recommended.
- Don't smoke. This is very important. Stopping smoking can lead to improvement within several months.
- Follow a low-glycaemic, low-dairy diet, and aim for ideal body weight. Note: profound weight loss can lead to skin folds—and these can exacerbate hidradenitis suppurativa because of friction.
- Wear loose fitting clothing to avoid friction.
- Hair removal and antiperspirants may be helpful.
- Wash with antiseptics, bleach baths or acne preparations to reduce skin carriage of commensal bacteria.
- Hydrogen peroxide solution and medical grade honey have been found to reduce malodour.
- For draining sinuses, apply simple dressings. Absorbent panty-liners are often a practical solution. Ask your doctor or nurse to suggest suitable bandages.
- If simple analgesics such as paracetamol (acetaminophen) are not effective, ask your doctor for help with pain control.
Treatment is required long term in many patients, as hidradenitis is a chronic disease.
- Topical anti-acne antibiotics such as clindamycin can be applied to affected areas, in combination with benzoyl peroxide to reduce bacterial resistance.
- Short course of oral antibiotics is used for acute abscesses (red, hot painful discharging lump) due to staphylococcal infection. Flucloxacillin or dicloxacillin are the most suitable, except in the case of penicillin allergy. Which antibiotic is used may depend on bacteriological cultures and antibiotic sensitivity.
- Prolonged courses (minimum 3 months) of tetracycline or metronidazole, cotrimoxazole, fluoroquinolones and dapsone are used mainly for their anti-inflammatory action.
- Six-to-twelve week courses of the combination of clindamycin and rifampicin is more effective than standard therapy and is often appropriate for severe cases. Doxycycline can replace clindamycin.
- Metformin, usually used in type 2 diabetes, is often beneficial when taken longterm, reducing the number and severity of inflamed nodules.
- Long-term oral contraceptive pill; those containing the antiandrogenic progesterones drospirenone or cyproterone acetate may be more effective than standard combined pills. Response takes 6 months or longer. These contraceptives are more suitable than progesterone-only pills or devices.
- The antiandrogenic medications spironolactone and finasteride have been reported to help some patients.
- Oral retinoids (vitamin A derivatives) for 6 to 12 months, especially isotretinoin, which are very effective for acne, may also help hidradenitis suppurativa. Acitretin is unsuitable for females of childbearing potential, but may be more effective than isotretinoin and long term remissions have been reported. Retinoids may be most useful in patients with comedones.
- Colchicine has been reported to help some patients with hidradenitis suppurativa.
- Systemic corticosteroids short-term, or intralesional corticosteroids (injections directly into the nodules), may reduce severe inflammatory lesions. Some patients take 3-day courses now and then for flare-ups.
- Other immunomodulatory treatments, including ciclosporin, methotrexate and azathioprine, have been successful in some patients with severe disease.
- In severe hidradenitis suppurativa, the use of biologics such as infliximab and adalimumab (TNFα inhibitors), may be worth considering. Adalimumab was approved by the European Medicines Agency in Jun 2015 and by the FDA in September 2015 for the treatment of hidradenitis suppurativa. Published data suggests higher doses are required to reduce the inflammatory component of hidradenitis suppurativa compared with doses usually prescribed for psoriasis. TNFα inhibitors are not currently funded by PHARMAC or registered for use in hidradenitis in New Zealand.
- Anakinra was reported to be remarkably affective in a patient with severe hidradenitis.
- Incision and drainage of abscesses – at the very painful pointing stage
- Scraping out nodules and abcesses (curettage)
- Wide local excision of persistent hidradenitis lumps
- Radical excisional surgery
- Laser hair removal and surface tissue ablation (de-roofing)
- van der Zee HH, Boer J, Prens EP, Jemec GB. The effect of combined treatment with oral clindamycin and oral rifampicin in patients with hidradenitis suppurativa. Dermatology. 2009;219(2):143-7. Epub 2009 Jul 8.
- Slade DEM, Powell BW, Mortimer PS. Hidradenitis suppurativa: pathogenesis and management. The British Association of Plastic Surgeons 2003; 56: 451-461
- Book: Textbook of Dermatology. Ed Rook A, Wilkinson DS, Ebling FJB, Champion RH, Burton JL. Fourth edition. Blackwell Scientific Publications.
- Buimer MG, Wobbes T, Klinkenbijl THG. Hidradenitis suppurativa British Journal of Surgery 2009:96;350-360
- Schrader AM, Deckers IE, van der Zee HH, Boer J, Prens EP. Hidradenitis suppurativa: a retrospective study of 846 Dutch patients to identify factors associated with disease severity. J Am Acad Dermatol. 2014 Sep;71(3):460-7.
- 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 GB. European S1 guideline for the treatment of hidradenitis suppurativa/acne inversa. J Eur Acad Dermatol Venereol. 2015 Apr;29(4):619-44. doi: 10.1111/jdv.12966. Epub 2015 Jan 30. PubMed PMID: 25640693.
On DermNet NZ:
- Hidradenitis suppurativa (German version, January 2010)
- Sebaceous adenitis
- Follicular occlusion syndrome
- Hidradenitis suppurativa US National Library of Medicine Genetics Home Reference
- Hidradenitis Suppurativa – Medline Plus
- The Hidradenitis Suppurativa Trust
- HS-USA Support Group
- Hidradenitis Suppurativa Foundation, Inc.
- Dermatologic Manifestations of Hidradenitis Suppurativa – Medscape Reference
- Hidradenitis Suppurativa – British Association of Dermatologists
- Patient information: Hidradenitis suppurativa (The Basics) – UpToDate (for subscribers)