Hidradenitis suppurativa

Hidradenitis suppurativa (also known as "Verneuil's disease"^[1]) is a skin disease that affects areas bearing apocrine sweat glands or sebaceous glands; such as the underarms, hands, ears, face, scalp, legs, groin and buttocks.^[2]:710

Overview

The non-contagious disease manifests as clusters of chronic abscesses, epidermoid cyst, sebaceous cysts, pilonidal cyst or multilocalised infections, which can be as large as baseballs or as small as a pea, that are extremely painful to the touch and may persist for years with occasional to frequent periods of inflammation, culminating in drainage of pus, often leaving open wounds that will not heal. Drainage provides some relief from severe, often debilitating, pressure pain. Flare-ups may be triggered by severe stress, perspiration, hormonal changes (such as monthly cycles in women), humid heat, and clothing friction. Persistent lesions may lead to scarring and the formation of sinus tracts, or tunnels connecting the abscesses or infections under the skin. At this stage, complete healing is usually not possible, and progression varies from person to person, with some experiencing remission anywhere from months to years at a time, others may worsen and require surgery in order to live comfortably. Wound dehiscence, a premature "bursting" open of a wound often complicates the healing process. Occurrences of bacterial infections and cellulitis (deep tissue inflammation) may occur at these sites. HS pain and depression can be difficult to manage.^[3]

HS often goes undiagnosed for years because patients are too ashamed to speak with anyone.^[3] When they do see a doctor, the disease is frequently misdiagnosed or prescribed treatments are ineffective, temporary and sometimes even harmful. There is no known cure nor any consistently effective treatment. Carbon dioxide laser surgery is currently considered the last resort for those who have advanced to its highest stage, where the affected areas are excised, and the skin is grafted. Surgery doesn't always alleviate the condition, however, and can be very expensive.

Several articles and clinics consider this disease as widely misdiagnosed, due to the misunderstanding of the causes and progression of the disease.^[4] HS is not the biblical stigmata, leprosy or caused by poor hygiene. HS is often called an 'orphan illness', due to little research being conducted on the disease at this time. Because HS is considered a rare disease, its incidence rate is not well known, but has been estimated as being between 1:24 (4.1%) and 1:600 (0.2%).^[5]

Other names for HS

Hidradenitis suppurativa has been referred to by multiple names in the literature, as well as in various cultures. Some of these are also used to describe different diseases, or specific instances of this disease.^[3]

• Acne conglobata - not really a synonym - this is a similar process but in classic acne areas of chest and back
• Acne Inversa (AI) - a proposed new term^[6][7] which has not gained widespread favour.^[8]
• Apocrine Acne - a misnomer, out-dated, based on the disproven concept that apocrine glands are primarily involved
• Apocrinitis - another misnomer, out-dated, based on the disproven concept that apocrine glands are primarily involved
• Fox-den disease - a catchy term not used in medical literature, based on the deep fox den / burrow - like sinuses
• Hidradenitis Supportiva - a misspelling
• Pyodermia fistulans sinifica - an older term, considered archaic now
• Velpeau's disease - commemorating the French surgeon who first described the disease in 1833
• Verneuil's disease - recognizing the French surgeon whose name is most often associated with the disorder as a result of his 1854-1865 studies^[9]

Stages

HS presents itself in three stages.^[3][10]

Causes

As this disease is poorly studied, the causes are controversial and experts disagree. However, potential indicators include:

• post-pubescent individuals are more likely to exhibit HS
• females are more likely than males
• Possible genetic predisposition among families of Sephardic Jewish, Italian, French, Dutch, Greek, Middle Eastern and Northern African Ancestry.
• Research is assessing possible relations with Hashimoto's Thyroiditis, Crohn's Disease, Rheumatoid Arthritis, and Squamous Cell Carcinoma.
• Plugged apocrine (sweat) gland or hair follicle
• excessive sweating
• bacterial infection
• sometimes linked with other auto-immune conditions^[11]
• androgen dysfunction
• genetic disorders that alter cell structure
• stress can bring on outbreaks
• being overweight makes it worse, however this condition is not caused by obesity and weight loss will improve but not cure it. Patients with more advanced cases may find exercise intolerably painful, which may increase the rate of obesity among sufferers.

The historical understanding of the disease is that there is a dysfunctional apocrine glands^[10] or dysfunctional hair follicles,^[12] possibly triggered by a blocked gland, creating inflammation, pain, and a swollen lesion. More recent studies imply there is an autoimmune component.^{[citation needed]}

HS is not caused by any bacterial infection—any infection is secondary—and is therefore not contagious. Most cultures done on HS lesions come back negative for bacteria, so antibiotics should be used only when a bacterial infection has been confirmed by a physician.^{[citation needed]}

Severe complications

In disease stage III, fistulas left undiscovered, undiagnosed, or untreated, can lead to the development of squamous cell carcinoma, a rare cancer, in the anus or other affected areas. ^[13][14] Other stage III chronic sequelae may also include anemia, multilocalised infections, amyloidosis, and arthropathy. Stage III complications have been known to lead to death, but clinical data is still uncertain.

Treatments

Treatments may vary depending upon presentation and severity of the disease. Due to the poorly-studied nature of this disease, the effectiveness of the drugs and therapies listed below is not yet clear, and patients should discuss all options with their doctor or dermatologist. Nearly a quarter of patients state that nothing relieves their symptoms.^[15] A list of treatments that are possible treatments for some patients is as follows.

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• Lifestyle
  • Changes in diet avoiding inflammatory foods, foods high in refined carbohydrates.
  • Warm compresses, hydrotherapy, balneotherapy
  • Icing the inflamed area daily until pain reduction is noticed

• Medication
  • Antibiotics orally.These are used for their anti-inflammatory properties rather than to treat infection. The most effective is a combination of Rifampicin [300 mg twice a day] and Clindamycin [300 mg twice a day] given concurrently for 2-3 months. This brings about remission in around three quarters of cases.[1]
  • Hexachlorophene shower with liquid soap like Phisohex, covering sores with Metrolotion after medicated showers.
  • Corticosteroid injections.
  • Vitamin A supplementation
  • Anti-androgen therapy
  • IV or subcutaneous infusion of anti-inflammatory (anti-TNF-alpha) drugs such as infliximab (Remicade), etanercept (Enbrel),^[16] and adalimumab.^[17] This use of the drugs is not currently Food and Drug Administration (FDA) approved and is somewhat controversial, and therefore may not be covered by insurance.
  • Acitretin
  • Zinc gluconate taken orally has been shown to induce remission
  • Chlorhexidine (Hibiclens) plus an antibiotic soap for cleansing the skin surface
  • Tetracycline
  • Tea Tree Oil & Tea Tree Oil Body Wash for cleansing the skin surface
  • Turmeric capsules orally or through topical application

• Surgery
  • Incision and drainage or lancing
  • Wide local excision (with or without skin grafting)
  • Laser surgery

• Radiation therapy

See also

• List of cutaneous conditions
• Hidradenitis

Footnotes

External links

• Medline: What is Hidradenitis Suppurativa?
• The Doctor's Doctor
• Hidradenitis Suppurativa (2004) Prof J. Revuz
• Hidradenitis Suppurativa Links
• Hidradenitis Suppurativa Support A support site for sufferers of Hidradenitis Suppurativa
• Hidradenitis Suppurativa Foundation A Foundation site for sufferers of Hidradenitis Suppurativa
• HS-USA HS-USA is a nonprofit public 501c3 charity incorporated in the State of Michigan, USA.
• Undiagnosed Illnesses Support Group For Undiagnosed Illnesses
• ***UK*** - Support Group for sufferers of Hidradenitus Suppurativa Hidradenitis Suppurativa Friends - Online forum support group in the United Kingdom