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Hidradenitis suppurativa is a chronic persistent or recurrent suppurative disease of unknown cause occurring in the apocrine follicles, usually affecting the groin and axillae and also other apocrine-bearing sites such as the breasts, perineum and buttocks. Follicular occlusion may lead to chronic relapsing inflammation, mucopurulent discharge and progressive scarring. Induration, ulceration and also sinus and fistula formation may occur.
- The prevalence in industrialised countries has been estimated to be in the region of 1%.
- Overall it occurs more often in women. Submammary, axillary, and inguinal involvement are more common in females, but perineal involvement is more common in men.
- Hidradenitis suppurativa is more common in white and Afro-Caribbean populations and is rare in people from Asia.
- Cigarette smoking.
- Lithium therapy.
- Possibly increased in patients with diabetes.
- Tends to improve during pregnancy and when taking the combined oral contraceptive pill (COCP); tends to relapse after pregnancy and when stopping the COCP.
- Usually starts at puberty but can present at any time from puberty until middle age and is variable in severity and distribution.
- Predominantly occurs in the axilla, groin, perineum, buttocks, scrotum, and the submammary regions.
- Early lesions are solitary, painful and pruritic nodules and multiple sites may be affected at the same time. They may persist for weeks or months. Any subcutaneous extension appears as indurated plaques. Episodes of acute cellulitis may occur.
- The nodules develop into pustules and eventually rupture with discharge of purulent material.
- Healing occurs with dense fibrosis.
- Recurrences tend to occur in the same region, leading to chronic sinus formation, with intermittent release of serous, purulent, or bloodstained discharge. Sinus formation and rupture may occur internally into adjacent structures as well as externally.
- Regional lymphadenopathy is usually absent.
Diagnosis is clinical but investigations may include:
- FBC: underlying anaemia associated with chronic disease.
- Blood glucose: identify associated diabetes.
- Microbiology swabs.
- CT/MRI scans: to map the extent of disease accurately prior to surgery.
- Cystic acne
- Pilonidal sinus
Hidradenitis suppurativa can be divided into the following three clinical stages (Hurley’s classification):
- Stage I: abscess formation, single or multiple without sinus tracts and scarring.
- Stage II: recurrent abscesses with sinus tracts and scarring; single or multiple widely separated lesions.
- Stage III: diffuse or almost diffuse involvement or multiple interconnected tracts and abscesses.
- Early lesions are usually treated by medical therapy, but long-standing disease usually requires surgery.
- Radiotherapy may be considered as a treatment option for some patients.
- Dermatology referral should be considered for patients with exacerbations that are frequent enough to cause them distress despite treatment and those with active disease and scarring in at least one site (Hurley stage II disease).
- Advice to patients should include good hygiene, using soaps, antiseptics and antiperspirants, warm compresses and wearing loose-fitting clothing.
- Advise weight reduction if obese, and smoking cessation.
- Beneficial activities include swimming.
- Antibiotics: a short course of antibiotics for two weeks; therapy will be governed by swab results but erythromycin, metronidazole, minocycline, clindamycin, cephalosporins and penicillins are all effective.
- Intralesional steroids: eg, triamcinolone; cause early lesions to resolve within 12-24 hours.
- Chronic relapsing stage:
- Long-term antibiotics (minimum 12 weeks): long-term erythromycin or tetracycline has been shown to reduce the relapse rate. The benefits may be lost after long-term use, and stopping therapy for a month and then restarting are recommended.
- High-dose oral steroids to reduce the inflammation may be required.
- Oestrogens: the COCP, particularly Dianette®, is often beneficial.
- Retinoids: acitretin appears to be an effective treatment for refractory disease.
- Dapsone is a treatment option for maintenance treatment in patients with moderate disease unsuitable for other treatments.
- Inhibitors of tumour necrosis factor alpha may be a treatment option for patients with severe disease resistant to other treatments.
- Only surgery can remove scarred areas. Surgery should be performed during a quiescent phase.
- Radical excision of the apocrine glands may produce temporary relief of symptoms, but the disease process may recur in residual glands in the surrounding area. Recurrence is more common in inadequate excision, obesity, continued smoking and locally macerated skin.
- Surgery may be extremely complex for extensive disease, with large areas of skin needing to be removed and problems with skin closure. Skin grafting or rotation flaps may be required. Infections and delayed healing are common.
- Limited surgical procedures include deroofing of lesions and carbon dioxide laser ablation.
- Fistula formation into the urethra, bladder, rectum, or peritoneum may occur but is uncommon.
- Chronic infection may lead to anaemia, hypoproteinaemia, amyloidosis and renal failure.
- Chronic malaise and depression.
- Scarring may lead to lymphatic obstruction and lymphoedema.
- Generalised arthropathy may occur in long-standing disease but is uncommon.
- Chronic sinuses occasionally result in the development of squamous cell carcinoma.
- Variable but without treatment tends to be a relentless progressive disease with acute exacerbations and remissions, leading to sinus tract formation and scarring.
- Early wide excision has been shown to be followed by a low recurrence rate.
Further reading & references
- Hidradenitis Suppurativa; DermIS (Dermatology Information System)
- Collier F, Smith RC, Morton CA; Diagnosis and management of hidradenitis suppurativa. BMJ. 2013 Apr 23;346:f2121. doi: 10.1136/bmj.f2121.
- Revuz J; Hidradenitis suppurativa. J Eur Acad Dermatol Venereol. 2009 Sep;23(9):985-98. doi: 10.1111/j.1468-3083.2009.03356.x.
- Revuz JE, Canoui-Poitrine F, Wolkenstein P, et al; Prevalence and factors associated with hidradenitis suppurativa: results from two J Am Acad Dermatol. 2008 Oct;59(4):596-601.
- Trombetta M, Werts ED, Parda D; The role of radiotherapy in the treatment of hidradenitis suppurativa: case report and review of the literature. Dermatol Online J. 2010 Feb 15;16(2):16.
- Boer J, Nazary M; Long-term results of acitretin therapy for hidradenitis suppurativa. Is acne inversa also a misnomer? Br J Dermatol. 2011 Jan;164(1):170-5. doi: 10.1111/j.1365-2133.2010.10071.x.
- Mandal A, Watson J; Experience with different treatment modules in hidradenitis suppuritiva: a study of 106 cases. Surgeon. 2005 Feb;3(1):23-6.
- Scheinfeld N; Hidradenitis suppurativa: A practical review of possible medical treatments based on over 350 hidradenitis patients. Dermatol Online J. 2013 Apr 15;19(4):1.
- Onderdijk AJ, van der Zee HH, Esmann S, et al; Depression in patients with hidradenitis suppurativa. J Eur Acad Dermatol Venereol. 2013 Apr;27(4):473-8. doi: 10.1111/j.1468-3083.2012.04468.x. Epub 2012 Feb 20.
- Alharbi Z, Kauczok J, Pallua N; A review of wide surgical excision of hidradenitis suppurativa. BMC Dermatol. 2012 Jun 26;12:9. doi: 10.1186/1471-5945-12-9.
Disclaimer: This article is for information only and should not be used for the diagnosis or treatment of medical conditions. EMIS has used all reasonable care in compiling the information but make no warranty as to its accuracy. Consult a doctor or other health care professional for diagnosis and treatment of medical conditions. For details see our conditions.
|Original Author: Dr Colin Tidy||Current Version: Dr Colin Tidy||Peer Reviewer: Dr Helen Huins|
|Last Checked: 19/11/2013||Document ID: 2261 Version: 22||© EMIS|