Hidradenitis suppurativa

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Hidradenitis suppurativa
Classification and external resources
ICD-10 L73.2
ICD-9 705.83
DiseasesDB 5892
eMedicine emerg/259  med/2717 derm/892
MeSH D017497

Hidradenitis suppurativa or HS is a skin disease that affects areas bearing apocrine sweat glands and hair follicles; such as the underarms, groin and buttocks. It is more commonly found in women and can be present under the breasts.

Contents

[edit] Overview

The disease manifests as clusters of chronic abscesses or boils, 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 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 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. Occurrences of bacterial infections and cellulitis (deep tissue inflammation) may occur at these sites. HS pain can be difficult to manage.[1]

HS often goes undiagnosed for years because patients are too ashamed to speak with anyone.[1] 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.

It is possible that there is genetic predisposition to the disease.[2] HS is not contagious, and is not affected nor caused by good or bad 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%).[3]

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.[1]

  • Acne conglobata - not really a synonym - this is a similar process but in classic acne areas of chest and back
  • Acne Inversa (AI) - a new term which has not found favour.[4]
  • 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 sinifica fistulans - an older term, considered archaic now, misspelled here
  • 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[5]

[edit] Stages

HS presents itself in three stages.[1][6]

Stage Characteristics
I Solitary or multiple isolated abscess formation without scarring or sinus tracts. (A few minor sites with rare inflammation; may be mistaken for acne.)
II Recurrent abscesses, single or multiple widely separated lesions, with sinus tract formation. (Frequent inflammations restrict movement and may require minor surgery such as incision and drainage.)
III Diffuse or broad involvement across a regional area with multiple interconnected sinus tracts and abscesses. (Inflammation of sites to the size of golf balls, or sometimes baseballs; scarring develops, including subcutaneous tracts of infection - see fistula. Obviously, patients at this stage may be unable to function.)

[edit] 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
  • genetic predisposition among families
  • plugged apocrine (sweat) gland or hair follicle
  • excessive sweating
  • bacterial infection
  • sometimes linked with other auto-immune conditions[7]
  • 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[6] or dysfunctional hair follicles,[8] 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]


[edit] Severe complications

Left undiscovered, undiagnosed, or untreated, the fistulas from severe stage-3 HS can lead to the development of squamous cell carcinoma in the anus or other affected areas. [9][10]

[edit] 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.[11] A list of treatments that are possible treatments for some patients is as follows.

[edit] Footnotes

  1. ^ a b c d "HS-USA :: What is Hidradenitis Suppurativa?". Retrieved on 2007-07-08.
  2. ^ Gao M, Wang PG, Cui Y, et al (2006). "Inversa acne (hidradenitis suppurativa): a case report and identification of the locus at chromosome 1p21.1-1q25.3". J. Invest. Dermatol. 126 (6): 1302–6. doi:10.1038/sj.jid.5700272. PMID 16543891, http://www.nature.com/jid/journal/v126/n6/abs/5700272a.html. 
  3. ^ "HS-USA :: Prevalence of Hidradenitis Suppurativa". Retrieved on 2007-07-08.
  4. ^ Scheinfeld N (2006). "Hidradenitis should not be renamed acne inversa". Dermatol. Online J. 12 (7): 6. PMID 17459292. 
  5. ^ Verneuil AS: Etudes sur les tumor de la peau. Arch Gen Med 1854; 94: 693.
  6. ^ a b DermNet acne/hidradenitis-suppurativa
  7. ^ ClinicalTrials.gov NCT00329823 Etanercept in Hidradenitis Suppurativa
  8. ^ "HSF - What is Hidradenitis Suppurativa? What is HS?". Retrieved on 2007-07-08.
  9. ^ Talmant JC, Bruant-Rodier C, Nunziata AC, Rodier JF, Wilk A (2006). "[Squamous cell carcinoma arising in Verneuil's disease: two cases and literature review]" (in French). Ann Chir Plast Esthet 51 (1): 82–6. doi:10.1016/j.anplas.2005.11.002. PMID 16488526. 
  10. ^ Short KA, Kalu G, Mortimer PS, Higgins EM (2005). "Vulval squamous cell carcinoma arising in chronic hidradenitis suppurativa". Clin. Exp. Dermatol. 30 (5): 481–3. doi:10.1111/j.1365-2230.2005.01875.x. PMID 16045671, http://www.blackwell-synergy.com/doi/abs/10.1111/j.1365-2230.2005.01875.x. 
  11. ^ "abscesses.org - About Hidradenitis Suppurativa". Retrieved on 2007-07-08.
  12. ^ Cusack C, Buckley C (2006). "Etanercept: effective in the management of hidradenitis suppurativa". Br. J. Dermatol. 154 (4): 726–9. doi:10.1111/j.1365-2133.2005.07067.x. PMID 16536817. 
  13. ^ Scheinfeld N (2006). "Treatment of coincident seronegative arthritis and hidradentis supprativa with adalimumab". J. Am. Acad. Dermatol. 55 (1): 163–4. doi:10.1016/j.jaad.2006.01.024. PMID 16781316. 

[edit] External links

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Diseases of the skin and subcutaneous tissue (integumentary system) (L, 680-709)
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