Hidradenitis suppurativa is a chronic (long-term) skin condition. The cause is unknown. Inflammation of the apocrine sweat gland-bearing areas leads to painful and recurrent boils and abscesses. Suppuration means formation or discharge of pus. These areas (commonly the armpits and groin) leak pus and are difficult to heal. Eventually, scarring occurs. Treatments include antibiotics, surgical drainage of the abscesses and, sometimes, surgical removal of the affected skin areas.
The skin and sweat glands
There are two different types of sweat gland in the skin on the human body. These are:
- Apocrine sweat glands.
- Eccrine (or merocrine) sweat glands.
Apocrine sweat glands
These are larger than the eccrine sweat glands, and found only in the skin on certain areas of the body. These areas include the underarms (axillae); under the breasts and around the nipples; and in the groin and genital region.
The main difference between apocrine and the more common eccrine sweat glands is that apocrine glands release their fluid (secretions) into the hair follicles, rather than directly on to the skin. The secretions are a thick, milky fluid, which can easily be turned into smelly body odour by bacteria. The secretions from apocrine glands does appear to have any function. It contains pheromones which are chemicals that are supposed to help humans find a mate!
The apocrine glands only start working at puberty, due to the action of the hormone changes that occur at this time.
Eccrine (merocrine) sweat glands
There are many more of these smaller sweat glands. They are found all over the body, with the largest numbers being found on the palms of the hands, the soles of the feet, and the forehead.
These sweat glands release sweat directly on to the skin surface through pores. They are located in the skin, but not as deep as the apocrine glands. This sweat is watery and clear, and contains various salts, and other waste chemicals the body needs to get rid of. The sweat helps to cool us down and is also secreted as an emotional response - for example, when anxious or stressed.
The nervous system controls both types of sweat glands. However, each type responds via different nerve fibres and different chemical messengers (neurotransmitters).
What is hidradenitis suppurativa?
Hidradenitis suppurativa is a chronic (long-term) inflammatory skin disease with recurrent boil-like lumps. These boils often get bigger, and turn into abscesses. The abscesses leak pus and become difficult to heal.
The problem affects only areas of the skin containing apocrine sweat glands.
Commonly, the problem affects the groin and armpits. Other areas are sometimes affected such as under the breasts, the vulva, the scrotum, the buttocks and the perineum. (The perineum is the area of skin between the anus and, either the vulva in a woman, or the scrotum, in a man.). Women tend to develop it more commonly in the armpits, groin and under the breasts. Men more commonly develop disease that affects the perineal and perianal (around the anus) skin.
The wounds caused by the boils and abscesses heal poorly with scarring. In severe cases, the pus tunnels down under the skin surface. The channels (or tunnels) formed are called sinus tracts. Multiple areas of hidradenitis can become linked under the skin surface, by a network of interconnected sinus tracts. This means the inflammation (and sometimes infection) travels deeper and becomes more widespread.
The eventually healed areas are full of thick scar tissue. The scarring can be as unsightly as the discharging wounds themselves.
Is hidradenitis suppurativa known by any other names?
Hidradenitis suppurativa is known by several other names, which can cause confusion. These are:
- Acne inversa
- Apocrine acne
- Acne conglobata
- Verneuil's disease
- Velpeau's disease
What causes hidradenitis suppurativa?
The cause is not well understood. It is thought to happen due to blockage of the hair follicles on the skin, or the sweat gland openings themselves. This blockage could be from sweat itself, or skin secretions, such as sebum from the sebaceous glands. The blocked sweat gland continues to make sweat. The sweat cannot escape on to the skin surface and so is forced deeper into surrounding tissue. Bacteria (germs) that normally live on the skin surface may have been trapped in the blocked gland or hair follicle. The bacteria can multiply in warm moist surroundings. As the sweat is forced back deeper into the tissues, it takes with it the bacteria. This leads to inflammation, and sometimes infection. This is how the hard boil-like lumps are thought to form to start with. As the problem gets worse, abscesses, which contain pus, develop.
It may also be that the sweat glands in some people don't develop correctly and completely. These glands might not allow the sweat they make to reach the skin surface. Instead, the sweat is trapped and travels into the surrounding tissues.
Who gets hidradenitis suppurativa?
Overall, about 1 in 100 people in the UK has hidradenitis suppurativa, meaning it is quite common. Many people will have very mild problems with it.
Hidradenitis suppurativa most commonly affects people aged between 20 and 40. It is three times more common in women than men. It is rare in Asian people, and far more common in Caucasians (white-skinned people) or Afro-Caribbean people.
Hidradenitis suppurativa does not occur in children, and develops only after puberty. This is because the sweat glands are activated by hormones called sex hormones, the levels of which increase during puberty. The problem tends to improve for women if they take the combined oral contraceptive pill (COCP) - the 'Pill', or if they are pregnant. It rarely occurs after the menopause. These things all suggest that hormones play a part in causing this disease.
The disease can run in families (about 1 in 3 cases), but the exact pattern of inheritance is not known.
Hidradenitis suppurativa is more common in obese people and in cigarette smokers. Obesity and smoking are not direct causes. However, they can be thought of as risk factors. Hidradenitis suppurativa also seems more common in people with acne and hirsutism (excessive abnormal hairiness, especially in women).
What are the signs and symptoms of hidradenitis suppurativa?
Hidradenitis suppurativa usually starts with a single inflamed boil-like nodule (firm raised skin lump). Sometimes this stage can result in itching, but usually there is discomfort.
The nodule either slowly disappears (between 10 and 30 days) or persists (remains) to become a suppurative (draining) abscess. Abscesses are usually very painful.
Eventually, healing occurs but the affected skin is permanently affected, leaving deep scarring. In more severe disease the affected areas spread. Either single or multiple abscesses occur. The formation of sinus tracts causes the overlying skin to feel hard and lumpy (indurated).
A staging system (Hurley's staging) can be used to describe the severity of the disease:
- Stage 1 - here there are either single or multiple areas affected, but the abscesses are separate from one another. There is no scarring or sinus tract formation.
- Stage 2 - involves recurrent abscesses which can be single or multiple. Although there are sinus tracts, the affected areas are usually widely separated.
- Stage 3 - generally large areas are affected with multiple interconnected sinus tracts and abscesses.
For some people, the disease is extremely distressing, with a constant succession of new nodules and abscesses forming as soon as older ones have finally healed.
Do I need any tests to diagnose hidradenitis suppurativa?
There are no tests used to diagnose hidradenitis suppurativa. The diagnosis is usually based on the typical signs and symptoms that a person may have.
Sometimes hidradenitis suppurativa is confused with other similar-looking skin conditions such as common boils, abscesses, skin infections and ingrowing hairs. Other diseases can cause sinus tracts, such as Crohn's disease. Tests might be needed to exclude these other conditions, though they often have many other symptoms.
Sometimes, if there are signs of infection, swabs can be taken. This is to see what bacteria (germs) are growing in the pus. This can help in deciding whether antibiotics (and which ones) should be used.
Occasionally, it might be helpful to test your blood for glucose (sugar) to make sure you do not have diabetes. This is because skin infections are more common in people with diabetes. Your doctor might also take blood to make sure you are not anaemic and to monitor the level of infection or inflammation.
Scans, such as CT scans, are not needed to diagnose the condition. They may, however, be used in very severe disease, to plan surgery as it is important to know where the sinus tracts go, and how deep they are.
What is the treatment for hidradenitis suppurativa?
Try to lose weight if you are obese, and stop smoking if you smoke.
Also, the following may help relieve some of your symptoms:
- Wear loose-fitting cotton clothing.
- Wash the affected areas carefully and gently, preferably using an antibacterial or antiseptic soap or shower gel. This is to try and get rid of bacteria on the skin. (Note that it is normal to have bacteria harmlessly living on the skin.)
- Avoid shaving affected areas, such as the underarms.
- Avoid using deodorants and antiperspirants if the underarms are affected, and avoid perfumes on affected areas too.
- You can use a hot flannel (as hot as you can bear), to hold against affected areas and encourage the abscesses to come to a 'point', so that they start draining. A tense hard abscess that has not burst is more painful than one where the pus is draining out.
- Try to minimise heat exposure and sweating. This might mean avoiding sitting next to the fireplace, or avoiding intense exercise in the gym.
It is difficult to control hidradenitis suppurativa with medical treatment. The aim is to catch the disease in its early stages, and treat and control milder forms of the disease. Medical treatment means using medicines, either on the skin (topically), or by mouth. Examples of medical treatment include:
- Topical anti-acne antibiotics and benzoyl peroxide. The antibiotics kill bacteria on the skin whilst the benzoyl peroxide is an antibacterial and also leads to drying of the skin. Benzoyl peroxide also encourages the skin to shed its dead surface, which can help stop the blockage of pores.
- Short courses of antibiotic tablets can be used when there are new abscesses. The aim is to try to stop the infection from spreading and to help the abscess heal more quickly. Generally a short course of antibiotics will last for two weeks.
- Prolonged courses of antibiotic tablets. These are usually used for their anti-inflammatory action.
- Trial of the COCP (the 'Pill') can be used. A trial of up to 12 months may be needed before deciding whether the skin has improved. Some COCPs (such as Dianette® or Yasmin®) might be better than others, as they counteract some of the more 'male' hormone effects such as skin oiliness and spots. Contraceptive pills are, of course, only suitable for women. Not all women can safely use the Pill, as contraceptive pills can have serious side-effects in some women. Your GP can discuss with you whether it is safe to use the Pill.
- Oral retinoids. Isotretinoin (Roaccutane®) is a retinoid. These are vitamin A-based medicines and should only be prescribed by a dermatologist (skin specialist). These medicines work by stopping the secretion of sebum from sebaceous glands. They also help the normal shedding of dead skin cells in the hair follicles, preventing pore blockage. Isotretinoin is often used in the treatment of acne but is less effective when used in hidradenitis suppurativa. It must not be taken in pregnancy due to the risk of birth defects. Common side-effects include extreme dryness of the skin and lips.
- Corticosteroid tablets (steroids), such as prednisolone, may be used in short courses to reduce inflammation. Long courses of steroids can cause serious side-effects such as osteoporosis (thinning of the bones), weight gain, high blood pressure, cataracts and mental health problems. (See separate leaflet called 'Steroid Tablets' for more information.)
- Medicines that affect the immune system have been used with some success. These medicines can only be prescribed by a specialist (such as a dermatologist) and your treatment must be carefully monitored. This is because there are potentially very serious side-effects such as renal (kidney) failure, and low blood counts (which can make you at risk of an overwhelming infection). Examples of drugs used are:
- Anti-TNF drugs such as infliximab (Remicade®).
- Ciclosporin (a drug that is also used to prevent rejection after organ transplant).
- Dapsone (usually used to treat leprosy; used in hidradenitis suppurativa for its anti-inflammatory action).
Some of these new medicines are very expensive. The NHS limits the use of these medicines to certain medical conditions, such as inflammatory bowel disease, rheumatoid arthritis and psoriasis. Use in hidradenitis suppurativa would be 'off-licence' and only likely to be available on a private basis.
Longstanding hidradenitis suppurativa usually requires surgery. Generally, this would be performed under general anaesthetic. The surgical procedure chosen depends on the grade or extent of the hidradenitis suppurativa. Surgical treatments include:
- Incision and drainage - this means lancing a tense, hard abscess and allowing the pus to drain out. This is most appropriate for grade 1 hidradenitis suppurativa, and a course of antibiotic tablets would usually be given afterwards.
- Wide-scale excision (removal) of affected areas - this can be used for grade 2 and 3 disease. For stage 2 disease, the sinus tracts are surgically removed. In stage 3 disease, the operation needs to be more extensive as the tracts and scarring go deeper and larger areas are involved. This means a lot of scarred, infected tissue has to be removed. Often skin grafts and other plastic surgery techniques are needed.
- Carbon dioxide laser treatment can be used as an alternative to conventional surgery, where available, and dependent on the severity of the disease. It is quite a new treatment. The diseased tissue is 'vapourised' leaving an open wound which is left to heal. Admission to hospital overnight is not normally needed.
Radiotherapy has been used to treat hidradenitis suppurativa, but this is not common.
Are there any complications from having hidradenitis suppurativa?
The main complication is scarring of the skin and deeper tissues. In severe cases, this can cause swelling of the arm (if the armpit was affected) or of the leg (if the groin was affected), called lymphoedema. This means that the fluid (lymph) drainage from the limb is affected and the fluid builds up, causing the swelling. It is a difficult problem to treat and cure; often, tight elastic compression garments have to be worn long-term.
Other complications include:
- General malaise (feeling unwell and tired).
- Chronic infection leading to problems such as anaemia, kidney failure and low levels of protein in the blood.
- Joint pains and inflammation (arthropathy).
- Skin cancer. This is very rare and has been reported in very severe long-term hidradenitis suppurativa.
- Fistula formation. A fistula is where the sinus tracts tunnel into other parts of the body, such as the bowel or bladder (this is extremely rare).
What is the prognosis (long-term outlook)?
The prognosis is very variable. Not everyone progresses from stage 1 to stage 3.
For many affected people, hidradenitis suppurativa is a painful and debilitating condition. It has a tendency to flare up regularly, gradually causing more problems. Deep scarring and sinus tract formation is not uncommon.
Some people have mild (stage 1) disease only. Early surgical treatment can (in some cases) cure the disease and stop it from returning. In rare cases, the condition goes away on its own without treatment.
Hidradenitis suppurativa is seldom a fatal condition unless there is an overwhelming infection in an immunocompromised person (that is, someone with an abnormal immune system).
The condition can prevent normal working and social activities (for example, swimming). Psychological problems are common, as are difficulties in sexual relationships. These problems can either be directly due to the pain and messiness of the condition, but also due to embarrassment and body image problems.
British Association for Hidradenitis Suppurativa
PO Box 550, Chatham, ME4 9AH Web: www.ba-hs.org.uk
A UK charity dedicated to raising awareness of hidradenitis suppurativa.
Hidradenitis Suppurativa Support
UK-based website providing information and support for people with hidradenitis suppurativa.
The British Association of Dermatologists
This is a professional organisation, but their website offers information for the general public about skin diseases including hidradenitis suppurativa.
Good information and pictures of hidradenitis suppurativa.
Further reading & references
|Original Author: Dr Tim Kenny||Current Version: Dr Katrina Ford|
|Last Checked: 23/08/2010||Document ID: 13488 Version: 1||© EMIS|
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.