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 (HS) is a skin disease that most commonly affects areas bearing apocrine sweat glands or sebaceous glands, such as the underarms, under the breasts, inner thighs, groin and buttocks.[1]

The non-contagious disease manifests as clusters of chronic abscesses, epidermoid cysts, sebaceous cysts, pilonidal cyst or multilocalised infections, which can be as large as tennis balls or as small as a pea. It can also start as a single abscess and once it pops, can make tracts of many more abscesses. These cysts can be 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. For unknown reasons, people with hidradenitis develop plugging or clogging of their apocrine glands.[2] HS causes chronic scarring and pus formation of the underarms (axilla) and groin/inner thigh areas. The simple procedure of incision and drainage provides some relief from severe, often debilitating, pressure and pain. Flare-ups may be triggered by perspiration, hormonal changes (such as monthly cycles in women), humidity and heat, and friction from clothing. Persistent lesions may lead to scarring and the formation of sinus tracts, or tunnels connecting the abscesses or infections under the skin.[3] 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.[citation needed] Occurrences of bacterial infections and cellulitis (deep tissue inflammation) may occur at these sites. Hidradenitis suppurativa pain and depression can be difficult to manage.[4]

HS often goes undiagnosed for years because patients are too ashamed to speak with anyone.[4] When patients see a physician or medical practitioner, the disease is frequently misdiagnosed. There is currently 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 does not always alleviate the condition, and may be very expensive[citation needed] for patients.

Several articles and clinics consider this disease as widely misdiagnosed, due to the misunderstanding of the causes and progression of the disease.[5] HS is not caused by poor hygiene.[citation needed] HS is often called an orphan illness due to little current research being conducted on the topic. Its incidence rate is not well established, but has been estimated as being between 1:24 (4.1%) and 1:600 (0.2%).[6]

Areas of involvement[edit]

HS patients (n=164, 121 females, 43 males) Female Male p (statistical significance)
Axillary 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 above table 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. (see Stages below)

Causes[edit]

Hidradenitis suppurativa is a poorly studied disease and its cause remains unknown. Experts disagree over proposed causes.[7]

HS occurs when hair follicles become plugged[vague]. Lesions occur in any body areas with hair follicle although intertriginous areas such as the axilla, groin, and perianal region are more commonly involved. This theory includes most of the following potentials indicators:[8]

The historical understanding of the disease is that there are dysfunctional apocrine glands[12] or dysfunctional hair follicles,[13] possibly triggered by a blocked gland, creating inflammation, pain, and a swollen lesion.

Triggering factors[edit]

There are a number of triggering factors that should be taken into consideration.

  • Obesity[14] is an exacerbating rather than a triggering factor,[15] through mechanical irritation, occlusion, and maceration.
  • Tight clothing,[14] and clothing made of heavy, non-breathable materials.
  • [16] Deodorants, depilation products, shaving of the affected area – their association with hidradenitis suppurativa is still an ongoing debate amongst researchers.[17]
  • Drugs, in particular oral contraceptives[18] (i.e., oral hormonal birth control; "the pill") and lithium.[19]
  • Hot and especially humid climates[20] (dry/arid climates often cause remission).
  • Gluten, packaged, processed foods. There has been new research that shows that eating a primal/caveman/gluten-free diet has shown successful remission in people with HS.
  • Nightshade family(tomatoes, peppers, eggplant, and potatoes) tend to cause flare ups, and/or mild allergic reactions.

Predisposing factors[edit]

Stages[edit]

HS presents itself in three stages.[4][12] Due to the large spectrum of clinical severity and the severe impact on quality of life, a reliable method for evaluating HS severity is needed. Evaluation should take into account the number, type and size of lesions, evolution, pain and repercussions for the quality of life of the patient.[citation needed] 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.[citation needed]

Hurley's staging system[edit]

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:[23]

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[edit]

The Sartorious staging system is more sophisticated than Hurley's. Sartorius et al. suggested that the Hurley system is not sophisticated enough to assess treatment effects in clinical trials during research. This classification allows for better dynamic monitoring of the disease severity in individual patients. The elements of this staging system are:[24]

  • 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 above categories, 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.[25]

Treatments[edit]

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 unclear, and patients should discuss all options with their physician or dermatologist. Nearly a quarter of patients state that nothing relieves their symptoms.[26] Possible treatments include:

Lifestyle[edit]

  • Changes in diet avoiding inflammatory foods, foods high in refined carbohydrates.
  • Warm compresses with distilled vinegar water, and taking hot baths with distilled white vinegar in the water [27] 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.[28]
  • Washing with benzoyl peroxide can be effective.[28]

Medication[edit]

  • Antibiotics- taken orally, these are used for their anti-inflammatory properties rather than to treat infection. Most effective is a combination of rifampicin and clindamycin given concurrently for 2–3 months. This brings about remission in around three quarters of cases.[29] A few popular antibiotics used to treat hidradenitis suppurativa include tetracycline, minocycline, and clindamycin.[30]
  • 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 ethinyl estradiol proved effective in randomized, controlled trials. Dosages reported have been very high.[31]
  • IV or subcutaneous infusion of anti-inflammatory (anti-TNF-alpha) drugs such as infliximab (Remicade), etanercept (Enbrel),[32] and adalimumab (Humira).[33] This use of these 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.[34]
  • 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 hidradenitis suppurativa.
  • Topical clindamycin has been shown to have an effect in double-blind placebo controlled studies.[35]
  • Topical resorcinol is a keratolytic agent that targets the follicular keratin plug and has been shown to have efficacy in several case series studies.[36]
  • Topical Sayman Salve zinc oxide and petrolatum: I have used for 45 years and always cures outbreaks completely. OTC

Radiation[edit]

Electron beam radiotherapy has been a successful treatment of hidradenitis, especially in Europe; it is not a common treatment option in most of the United States, as radiation oncologists generally refuse to treat patients with non-malignant diseases because of the potential for secondary radiation-induced tumors in the long term.

Surgery[edit]

When the process becomes chronic, wide surgical excision is the procedure of choice. Wounds in the affected area do not heal by secondary intention, and immediate application of a split thickness skin graft is more appropriate.[8]

Laser Hair Removal[edit]

The 1064 nanometer wavelength laser for hair removal aids in the treatment of HS.[37]

Prognosis[edit]

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.[38][39] Other stage III chronic sequelae may also include anemia, multilocalized infections, amyloidosis, and arthropathy. Stage III complications have been known to lead to death, but clinical data is still uncertain.

Potential complications[edit]

History[edit]

  • In 1839, Velpeau identified and described hidradenitis suppurativa.[46]
  • In 1854, Verneuil described hidradenitis suppurativa as "Hidrosadénite Phlegmoneuse". This is how HS obtained its alternate name "Verneuil's disease".[47]
  • In 1922, Schiefferdecker hypothesized a pathogenic link between "Acne inversa" and human apocrine sweat glands.[48]
  • 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 abscedens et suffodiens".[49] 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".[50] Plewig and Kligman's research follows in Pillsbury's footsteps, offering explanations of the symptoms associated with hidradenitis suppurativa.
  • In 1989, Plewig and Steger's research led them to rename hidradenitis suppurativa, calling it "Acne Inversa" – which is not still used today in medical terminology; although some individuals still use this outdated term.[51]

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 pathogenicassociation 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".

Author Year Findings
Velpeau 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

Other names[edit]

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.[4]

  • 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[52][53] which has not gained widespread favour.[54]
  • 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 term not used in medical literature, based on the deep fox den / burrow – like sinuses
  • Hidradenitis Supportiva – a misspelling
  • Pyodermia fistulans significa – now considered archaic
  • 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[55]

Dermatohistological view of hidradenitis suppurativa[edit]

Author Year Major Features
Plewig & Steger[51] 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[56] 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[57] 1996 Primary inflammation of the follicular infundibulum. Apocrine sweat glands are secondarily involved.

References[edit]

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External links[edit]