Hidradenitis suppurativa

Synonyms

1

Overview

Hidradenitis suppurativa (HS) is a chronic skin disease characterized by recurrent, painful, boil-like lumps (nodules) under the skin. HS affects the areas around skin folds (e.g., armpits, groin, and breasts) and where apocrine glands (a form of sweat gland) and hair follicles are found. It is not contagious, but it is recurrent. It typically manifests as a single boil-like, pus-filled abscess or hard sebaceous lumps (lumps composed of sebum, or oil, which is excreted by the sebacous glands associated with hair follicles) and may progress to painful, deep-seated, inflamed clusters of lesions with chronic seepage involving significant scarring. In most cases, the cause of HS is unknown. It is likely that it results from a combination of genetic and environmental factors. Some cases of HS have been associated with specific genes, including NCSTN, PSEN1, and PSENEN. 
Hidradenitis suppurativa tends to start after puberty, persist for years and worsen over time. Early diagnosis and treatment can help manage the symptoms and prevent new lesions from developing.

Symptoms

Signs and symptoms of hidradenitis suppurativa include:

  • Blackheads. Small pitted areas of skin containing blackheads — often appearing in pairs or a "double-barreled" pattern — are a common feature.
  • Red, tender bumps. These bumps, or lesions, often enlarge, break open and drain pus. The drainage may have an unpleasant odor. Itching, burning and excessive sweating may accompany the bumps.
  • Painful, pea-sized lumps. These hard lumps, which develop under the skin, may persist for years, enlarge and become inflamed.
  • Leaking bumps or sores. These open wounds heal very slowly, if at all, often leading to scarring and the development of tunnels under the skin.

Hidradenitis suppurativa commonly occurs in areas near hair follicles where there are many oil and sweat glands; places like the armpit, groin and anal area. It can also occur in areas where skin rubs together, including the inner thighs, under the breasts, and between the buttocks.

This condition most often presents after puberty and can persist for years, worsening over time. Three stages of disease have been described which progress from single or localized accesses accompanied by itching or discomfort, to recurrent abscesses that occur in multiple locations, to widespread severe disease that can restrict movement, obstruct lymph drainage and lead to social isolation.

Causes

Hidradenitis suppurativa develops when hair follicles become blocked and inflamed. The underlying cause is unknown why this blockage occurs. Factors that may play a role include hormones, metabolic syndrome, genetics, an irregular immune system response, smoking and excess weight. 

A family history of HS is reported in approximately 1/3 patients. Researcher suspect that HS is inherited or passed down in an autosomal dominant manner. This means that if an individual inherits HS, they have a 50% chance to pass it to the next generation. These risks may be lower, as there are likely other influences that impact one's likelihood of developing HS. Researchers are currently trying to identify which genes may be implicated in HS. Some cases have been found to result from mutations in the NCSTN, PSEN1, PSENEN gene. More studies are needed to determine whether other genes might be involved.

Prevention

Not really known but some factors that may increase your risk may include:

  • Age. Hidradenitis suppurativa most commonly occurs in women between the ages of 20 and 29. An early age of onset may be associated with developing more widespread disease.
  • Your sex. Women are more likely to develop hidradenitis suppurativa than are men.
  • Family history. A tendency to develop hidradenitis suppurativa can be inherited.
  • Having certain other conditions. Hidradenitis suppurativa can be associated with several other conditions, including arthritis, severe acne, obesity, inflammatory bowel disease, Crohn's disease, metabolic syndrome and diabetes.
  • Smoking. Smoking tobacco has been linked to hidradenitis suppurativa

Diagnosis

Diagnosis can usually be made by examining the affected skin. If pus or drainage is present, your doctor might send a sample of the fluid to a laboratory for testing.

The Genetic Testing Registry (GTR) provides information about the genetic tests for this condition. The intended audience for the GTR is health care providers and researchers. Patients and consumers with specific questions about a genetic test should contact a health care provider or a genetics professional.

Prognosis

Mild hidradenitis suppurativa may be treated with only self-care measures. But self-care is also an important complement to any medical treatment you may be getting. The following suggestions may help relieve discomfort and speed healing of sores:

  • Follow a daily skin-care routine. Gently wash your body with a nonsoap cleanser such as Cetaphil. Use only your hands, not washcloths, loofahs or other items that might irritate the skin. If odor is a concern, try an antibacterial body wash. Then apply an over-the-counter antibiotic cream. It might also help to apply extra absorbent powder or zinc oxide. Using antiperspirants may help keep the skin dry. Stop using any product that irritates your skin.
  • Manage your pain. Gently applying a wet, warm washcloth, teabag or other sort of compress can help reduce swelling and ease pain. Keep it on for about 10 minutes. Ask your doctor to recommend the most appropriate pain reliever. And talk with your doctor about how to properly dress and care for your wounds at home.
  • Avoid tight clothes and irritating products. Wear loose, lightweight clothes to reduce friction. Some women find that using tampons rather than sanitary pads causes less friction with the skin. Use detergents and other products that are free of perfumes, dyes and enzymes.
  • Avoid injuring the skin. For example, don't squeeze the pimples and sores. And stop shaving affected skin.
  • Keep a healthy weight and stay active. Not being at a healthy weight can make symptoms worse. Try to find activities that don't irritate your skin.
  • Consider altering your diet. In an informal study, 47 people with hidradenitis suppurativa gave up dairy products and processed sugar and flour. Of those, 83 percent experienced reduced symptoms. Also, a study reported on 12 people being treated for hidradenitis suppurativa who avoided beer and other foods containing brewer's yeast or wheat. They all saw their symptoms clear up within a year.
  • Avoid all tobacco products. If you smoke, try to quit. Smoking and other tobacco use may play a role in making hidradenitis suppurativa worse.

Treatment

  • Initial treatments may involve conservative measures such as warm baths, hydrotherapy, and topical cleansing agents to reduce bacterial loads.
  • Acute painful skin lesions may be treated with corticosteroids (e.g., prednisone) or anti-inflammatory pills (e.g., Celebrex, Advil, Naproxen, and others).
  • Although not proven to be effective, antibiotics are often the mainstay of medical treatment, especially for lesions suspected of being super infected.
  • Affected individuals may also be treated with oral contraceptives or other medications that address a possible hormonal cause.
  • Oral retinoids such as isotretinoin have also been used.
  • Other treatments that have been used with limited success include cisplatin, methotrexate, 5-alpha reductase inhibitors, and TNF-alpha inhibitors.
  • In advanced cases, affected individuals may undergo surgery (e.g., laser therapy or photodynamic therapy), but recurrences of HS are not uncommon.
  • Targeted therapy:
    Adalimumab ( Humira) is FDA-approved for the indication: Treatment of moderate to severe hidradenitis suppurativa (Hurley stage 2 and Hurley stage 3 disease)
  • Alternative medicine: Zinc supplements taken daily may help reduce inflammation and prevent new outbreaks.