Hidradenitis suppurativa

Infobox_Disease
Name = Hidradenitis suppurativa


Caption =
DiseasesDB = 5892
ICD10 = ICD10|L|73|2|l|60
ICD9 = ICD9|705.83
ICDO =
OMIM =
MedlinePlus =
eMedicineSubj = emerg
eMedicineTopic = 259
eMedicine_mult = eMedicine2|med|2717 eMedicine2|derm|892
MeshID = 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.

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.cite web |url=http://www.hs-usa.org/hidradenitis_suppurativa.htm |title=HS-USA :: What is Hidradenitis Suppurativa? |accessdate=2007-07-08 |format= |work=]

HS often goes undiagnosed for years because patients are too ashamed to speak with anyone. 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. [cite journal |author=Gao M, Wang PG, Cui Y, "et al" |title=Inversa acne (hidradenitis suppurativa): a case report and identification of the locus at chromosome 1p21.1-1q25.3 |journal=J. Invest. Dermatol. |volume=126 |issue=6 |pages=1302–6 |year=2006 |pmid=16543891 |doi=10.1038/sj.jid.5700272 |url=http://www.nature.com/jid/journal/v126/n6/abs/5700272a.html] 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%). [cite web |url=http://hs-usa.org/pub/articles/prevalence.htm |title=HS-USA :: Prevalence of Hidradenitis Suppurativa |accessdate=2007-07-08 |format= |work=]

;Other names for HSHidradenitis 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.

*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. [cite journal |author=Scheinfeld N |title=Hidradenitis should not be renamed acne inversa |journal=Dermatol. Online J. |volume=12 |issue=7 |pages=6 |year=2006 |pmid=17459292 |doi=]
*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 [Verneuil AS: Etudes sur les tumor de la peau. Arch Gen Med 1854; 94: 693.]

tages

HS presents itself in three stages.DermNet|acne/hidradenitis-suppurativa]

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 [ClinicalTrialsGov|NCT00329823|Etanercept in Hidradenitis Suppurativa]
* 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.

The historical understanding of the disease is that there is a dysfunctional apocrine glands or dysfunctional hair follicles, [cite web |url=http://www.hs-foundation.org/abouths/what.htm |title=HSF - What is Hidradenitis Suppurativa? What is HS? |accessdate=2007-07-08 |format= |work=] possibly triggered by a blocked gland, creating inflammation, pain, and a swollen lesion. More recent studies imply there is an autoimmune component.Fact|date=February 2007

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.Fact|date=February 2007

Progression

During "flare-up" periods, the disease can be expected to take a predictable course, as described below.

1). The early stage is characterized by comedo formation (arrowhead). Note that the apocrine glands are unremarkable (arrow). Sections were stained with hematoxylin-eosin; magnification: ×13 The disease process starts with follicular hyperkeratosis and dilatation of the follicular infundibula evolving into comedones, comparable to those observed in acne vulgaris (Fig. 1).

2). (a) Continuing dilatation of the follicular infundibulum leads to rupture into the surrounding dermis evoking an acute inflammatory infiltrate (arrowhead). The apocrine glands (arrows) are not involved. (b) Higher magnification of ruptured comedo. (c) Site of rupture (arrow) in another comedo. Sections were stained with hematoxylin-eosin; magnification: (a) ×4; (b) ×13; (c) ×165 At this time, the apocrine glands are not involved. Eventually the dilated follicular infundibulum ruptures, typically at the lower portion, and the content spills into the surrounding dermis, evoking an acute inflammatory response in the immediate vicinity of the rupture site (Fig. 2a–c).

3). (a)Over time, a granulomatous infiltrate (arrows) replaces the neutrophilic infiltrate and the process may remain confined. (b) Higher magnification of granulomatous infiltrate. Sections were stained with hematoxylin-eosin; magnification: (a) ×14; (b) ×162 Again, the apocrine glands do not show any signs of involvement nor an indication that they are the anatomical starting point of the sequence of events. If the inflammation remains confined to the immediate vicinity of the hair follicle, over time the initially neutrophilic infiltrate subsides and is gradually replaced by a granulomatous one, often with the addition of multinucleated foreign body giant cells (Fig. 3a–b).

4). (a) An abscess develops, if the inflammation following rupture of the comedo is more florid. Note that the apocrine glands are not involved (arrow). (b) Higher magnification of apocrine glands (arrow) in (a). Sections were stained with hematoxylin-eosin; magnification: (a) ×21; (b) ×83 If, however, the acute inflammatory response following rupture is more florid, a large abscess develops which may extend into the subcutaneous tissue (Fig. 4a).

5). (a) Only if the inflammation spreads further do the apocrine glands become involved (arrow). (b) Then, neutrophils invade the glandular lumina (arrow) and the glands are destroyed. Sections were stained with hematoxylin-eosin; magnification: (a) ×21; (b) ×83 Apocrinitis only evolves by extension of the inflammatory process, leading to destruction of apocrine glands (Fig. 5a–b).

6). Naked hair shafts may be the only indication that the process started from the hair follicle. Sections were stained with hematoxylin-eosin; magnification: ×42 Apocrine glands located further away from the extending abscess are morphologically unremarkable. When extensive tissue destruction has ensued, naked hair shafts, surrounded by an inflammatory infiltrate, are often the only indication that the process started from the hair follicle (Fig. 6).

7). Sinus tracts are a late sign of the disease. Sections were stained with hematoxylin-eosin; magnification: ×4. In an attempt of the tissue to confine the inflammatory reaction and to prevent further spread, remnants of the hair follicle epithelium proliferate and sinus tracts form, often surrounded by fibrosis (Fig. 7).

8). Figure 8 When they rupture, the disorder enters a vicious cycle. Sections were stained with hematoxylin-eosin; magnification: ×13 The sinus tracts communicate with the surface. Upon bacterial superinfection, they rupture and the process becomes self-maintaining and enters into a vicious cycle (Fig. 8). Sinus tract formation is the main reason for the chronicity of the disease.

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. [cite journal |author=Talmant JC, Bruant-Rodier C, Nunziata AC, Rodier JF, Wilk A |title= [Squamous cell carcinoma arising in Verneuil's disease: two cases and literature review] |language=French |journal=Ann Chir Plast Esthet |volume=51 |issue=1 |pages=82–6 |year=2006 |pmid=16488526 |doi=10.1016/j.anplas.2005.11.002 |url=] [cite journal |author=Short KA, Kalu G, Mortimer PS, Higgins EM |title=Vulval squamous cell carcinoma arising in chronic hidradenitis suppurativa |journal=Clin. Exp. Dermatol. |volume=30 |issue=5 |pages=481–3 |year=2005 |pmid=16045671 |doi=10.1111/j.1365-2230.2005.01875.x |url=http://www.blackwell-synergy.com/doi/abs/10.1111/j.1365-2230.2005.01875.x]

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. [cite web |url=http://abscesses.org/hidradenitisSuppurativa/content/view/22/33/ |title=abscesses.org - About Hidradenitis Suppurativa |accessdate=2007-07-08 |format= |work=] A list of treatments that are possible treatments for some patients is as follows.

* lifestyle
** changes in diet.Fact|date=December 2007
** warm compresses, baths (to induce drainage)

* medication
** Showering in Hexachlorophene liquid soap like Phisohex, covering sores with Metrolotion after medicated showers.
** intralesional corticosteroid injections (to reduce inflammation) Usually Kenalog.
** oral antibiotics (to treat inflammation and bacterial infection)
** isotretinoin (Accutane), a prescription-only oral acne treatment (benefits for HS are very controversial, but it is generally considered to be ineffectiveFact|date=April 2008)
** anti-androgen therapy
** sub-cutaneous injection or IV infusion of anti-inflammatory (anti-TNF-alpha) drugs such as infliximab (Remicade), etanercept (Enbrel), [cite journal |author=Cusack C, Buckley C |title=Etanercept: effective in the management of hidradenitis suppurativa |journal=Br. J. Dermatol. |volume=154 |issue=4 |pages=726–9 |year=2006 |pmid=16536817 |doi=10.1111/j.1365-2133.2005.07067.x] and adalimumab. [cite journal |author=Scheinfeld N |title=Treatment of coincident seronegative arthritis and hidradentis supprativa with adalimumab |journal=J. Am. Acad. Dermatol. |volume=55 |issue=1 |pages=163–4 |year=2006 |pmid=16781316 |doi=10.1016/j.jaad.2006.01.024] 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

* surgery
** incision and drainage or lancing
** wide local excision (with or without skin grafting), or laser surgery
** radiotherapy

Footnotes

External links

* [http://www.nlm.nih.gov/medlineplus/hidradenitissuppurativa.html Medline: What is Hidradenitis Suppurativa?]
* [http://www.thedoctorsdoctor.com/diseases/hidradenitis_suppurativa.htm The Doctor's Doctor]
* [http://www.orpha.net/data/patho/GB/uk-hidradenitis-suppurativa.pdf Hidradenitis Suppurativa (2004) Prof J. Revuz]
* [http://www.frontiernet.net/~ruthb/HidradenitisSuppurativa.html Hidradenitis Suppurativa Links]
* [http://www.hs-support.uni.cc Hidradenitis Suppurativa Support] A support site for sufferers of Hidradenitis Suppurativa
* [http://www.hs-foundation.org/ Hidradenitis Suppurativa Foundation ] A Foundation site for sufferers of Hidradenitis Suppurativa