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 (also known as "Verneuil's disease"[1], "Pyoderma fistulans significa,"[1] and "Acne inversa"[1]) is a skin disease that most commonly affects areas bearing apocrine sweat glands or sebaceous glands, such as the underarms, breasts, inner thighs, 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 incision and drainage of pus, often leaving open wounds that will not heal. The simple procedure of incision and 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), humidity and 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, while others may worsen and require multiple surgeries 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 or medical practitioner, 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 neither the biblical stigmata, leprosy nor 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]

Areas of Involvement in Men and Women with Hidradenitis Suppurativa[6]

HS Patients (n=164, 121 females, 43 males) Female Male p (Statistical Significance)
Axillae 70 (58%) 30 (70%) NS (not significant)
Mammary and inter-mammary 31 (26%) 2 (5%) 0.006
Inguino-femoral 111 (92%) 32 (74%) 0.007
Perianal and Perineal 40 (33%) 24 (56%) 0.01
Buttocks 30 (25%) 21 (50%) 0.006
  • Note that for the study involved in the Table above was a personal series of 164 patients. Of the 164 patients with HS, 76% were in Hurley's Stage I, 20% were in Hurley's Stage II, and 4% were in the final phase of the disease, Hurley's Stage III.


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[7][8] which has not gained widespread favour.[9]
  • Apocrine Acne - a misnomer, out-dated, based on the disproven concept that apocrine glands are primarily involved. Though many do suffer with apocrine gland infection, so thought should be given to using HS with subtext re: glands 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[10]

Historical Overview of Hidradenitis Suppurativa

  • In 1839, the first description of Hidradenitis Suppurativa was identified and published by Velpeau[11].
  • In 1854, Verneuil described Hidradenitis Suppurativa as "Hidrosadénite Phlegmoneuse". This is how HS obtained it's alternate name "Verneuil's disease"[12].
  • In 1922, Schiefferdecker hypothesized a pathogenic link between "Acne inversa" and human apocrine sweat glands[13].
  • In 1956, Pillsbury wrote and published a medical journal article discussing Hidradenitis Suppurativa, describing the disease's main characteristics, dubbing them the "Acne triad: hidradenitis suppurativa,perifolliculitis capitis abscendens et suffodiens"[14]. Pillsbury's research study was one of the first peer-reviewed journal articles to appear publicly with many details of Hidradenitis Suppurativa, which are still used and relied on today in the medical realm of research on this disease.
  • In 1975, Plewig and Kligman, following Pillsbury's research path, modified the "Acne triad", replacing it with the "Acne tetrad: acne triad, plus pilonidal sinus"[15]. Plewig and Kligman's research follows in Pillsbury's footsteps, offering explanations of the symptoms associated with Hidradenitis Suppurativa.
  • Finally in 1989, Plewig and Steger's research led them to rename Hidradenitis Suppurativa, calling it "Acne Inversa" - which not still used today in medical terminology; although some individuals still use this outdated term[16].


A surgeon from Paris, Velpeau described an unusual inflammatory process with formation of superficial axillary, sub-mammary and perianal abscesses in 1839. One of his colleagues also located in Paris, named Verneuil, coined the term “hidrosadénite phlegmoneuse” approximately 15 years later. This name for the disease reflects the former pathogenetic model of acne inversa, which is considered inflammation of sweat glands as the primary cause of Hidradenitis Suppurativa. In 1922 Schiefferdecker suspected a pathogenic association between acne inversa and apocrine sweat glands. In 1956 Pillsbury postulated follicular occlusion as the cause of acne inversa, which they grouped together with acne conglobata and perifolliculitis capitis abscendens et suffodiens (dissecting cellulitis of the scalp) as the "acne triad". Plewig and Kligman added another element to their acne triad, pilonidal sinus. Plewig et al. noted that this new "acne tetrad" includes all the elements found in the original "acne triad", in addition to a fourth element, pilonidal sinus. In 1989 Plewig and Steger introduced the term "acne inversa", indicating a follicular source of the disease and replacing older terms such as "Verneuil disease".

Historical View of Hidradenitis Suppurativa

Author Year Findings
Valpeau 1839 First description of the Hidradenitis Suppurativa
Verneuil 1854 "Hidrosadénite Phlegmoneuse"
Pillsbury 1956 Acne triad (hidradenitis suppurativa, perifolliculitis capitis abscendens et suffodiens)
Plewig & Kligman 1975 Acne tetrad (acne triad + pilonidal sinus)
Plewig & Steger 1989 Acne inversa

Dermatohistological View of Hidradenitis Suppurativa

Author Year Major Features
Plewig & Steger[16] 1989 Initial hyperkeratosis of the follicular infundibulum. Bacterial super-infection and follicle rupture. Granulomatous inflammatory reaction of the connective tissue. Apocrine and eccrine sweat glands secondarily involved.
Yu & Cook[17] 1990 Cysts and sinus tracts lined with epithelium, in part with hair shafts. Inflammation of apocrine sweat glands only if eccrine sweat glands and hair follicles are also inflamed.
Boer & Weltevreden[18] 1996 Primary inflammation of the follicular infundibulum. Apocrine sweat glands are secondarily involved.

Stages

HS presents itself in three stages.[3][19] Due to the large spectrum of clinical severity, to severe repercussions on quality of life and to the variety of treatments available, a reliable method for evaluating HS severity is required. It should take into account the number, type and size of lesions, evolution, pain and repercussions for the quality of life of the patient. Such a comprehensive instrument does not yet exist, but there have been two successful attempts proposed to classify patients with HS according to the severity of their disease.

Hurley's Staging System[20]

This is historically the first classification system proposed, and is still in use for the classification of patients with skin/dermatologic diseases (i.e. psoriasis, HS, acne). Hurley separated patients into three groups based largely on the presence and extent of cicatrization and sinuses. It has been used as a basis for clinical trials in the past and is a useful basis to approach therapy for patients. These three stages are based on Hurley's staging system, which is simple and relies on the subjective extent of the diseased tissue the patient has. Hurley's three stages of Hidradenitis Suppurativa are as follows:

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 inflammation 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.)

Sartorious Staging System[21]

The Sartorious staging system is more sophisticated than Hurley's, and is likely to supplant it as a means for conducting clinical trials during research. Sartorius et al. have suggested that the Hurley system is not sophisticated enough to assess treatment effects in clinical trials. The need for uniform outcome variables when reporting treatment effects has led to the proposition of a score by Sartorious and colleagues. This classification allows for better dynamic monitoring of the disease severity in individual patients and therefore forms a complimentary system to the Hurley classification. They suggest a system that incorporates several of the involved areas of consideration:

  • Anatomic regions involved (axilla, groin gluteal or other region or infra-mammary region left or right)
  • Number and types of lesions involved (abscesses, nodules, fistulas, scars, points for lesions of all regions involved)
  • The distance between lesions, in particular the longest distance between two relevant lesions (i.e. nodules and fistulas in each region or size if only one lesion present)
  • The presence of normal skin in between lesions (i.e. are all lesions clearly separated by normal skin?)

Points are accumulated in each of the mentioned categories above, and added to give both a regional and total score. In addition, the authors recommend adding a visual analog scale for pain or using the dermatology life quality index (DLQI, or the Skindex) when assessing HS[22]. This system will likely be the basis of most future clinical trials and research studies.

Causes

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

The historical understanding of the disease is that there is a dysfunctional apocrine glands[19] or dysfunctional hair follicles,[25] 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 elements.

Triggering Factors

There are a number of triggering factors that should be taken into consideration, as it is advisable to avoid such triggers.

Predisposing Factors

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.[34][35] 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.

Potential Complications

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

  • Lifestyle
    • Changes in diet avoiding inflammatory foods, foods high in refined carbohydrates.
    • Warm compresses[43], hydrotherapy, balneotherapy
    • Icing the inflamed area daily until pain reduction is noticed
    • Weight loss in overweight and obese patients, as well as smoking cessation can improve or even alleviate many symptoms of Hidradenitis suppurativa: Obese and overweight patients should be helped to lose weight[44] in all cases of HS regardless of which stage patient may be in.
  • Medication
    • Antibiotics Taken 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.[45] A few popular anitbiotics used to treat HS include tetracycline, minocycline, and clindamycin.
    • Corticosteroid injections. Also known as intralesional steroids: can be particularly useful for localized disease, if the drug can be prevented from escaping via the sinuses.
    • Vitamin A supplementation
    • Anti-androgen therapy: hormonal therapy with cyproterone acetate and ethinyloestadiol proved effective in randomized control trials. It is of note that dosages reported have been very high[46].
    • IV or subcutaneous infusion of anti-inflammatory (anti-TNF-alpha) drugs such as infliximab (Remicade), etanercept (Enbrel),[47] and adalimumab.[48] 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.
    • Zinc gluconate taken orally has been shown to induce remission. Recommended dose is at least 90 mg/3 times daily.[citation needed]
    • Chlorhexidine (Hibiclens) plus an antibiotic soap for cleansing the skin surface. Hexachlorophene shower with liquid soap like Phisohex, covering sores with Metrolotion after medicated showers. These are considered to be general measures, and are the foundation of any good medical treatment and management plans for HS.
    • Turmeric capsules orally or through topical application. The active ingredient in the tumeric spice is curcumin.[citation needed]
    • Infliximab: a chimeric monoclonal anti-TNF antibody has demonstrated efficacy in treating HS[43].
    • Topical clindamycin has been shown to have an effect in double-blind placebo controlled studies[49].

References

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External links

bs:Hidradenditis de:Acne inversa fr:Hidrosadénite nl:Hidradenitis suppurativa pl:Ropnie mnogie pach pt:Hidrosadenite sl:hidradenitis suppurativa

uk:Гідраденіт
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