The cysts seen in HS can be extremely tender and may persist for years with interspersed periods of inflammation, culminating in incision and drainage of pus from the cyst. This process often forms open wounds that will not heal. For unknown reasons, people with HS develop plugging or clogging of their apocrine glands. HS causes chronic scarring and pus formation of the underarms, groin, and inner thighs. The simple procedure of incision and drainage may provide symptomatic relief. HS flares may be triggered by perspiration, hormonal changes (such as those seen with the menstrual cycle), 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. At this stage, complete healing is usually not possible, and progression is variable, with some experiencing remission for months to years at a time, while others may worsen and require multiple surgeries. Bacterial infections and cellulitis (deep tissue inflammation) are other common complications of HS. Hidradenitis suppurativa pain and depression can be difficult to manage.
HS often goes undiagnosed for years due to a delay in seeking treatment and is frequently misdiagnosed. There is currently no known cure nor any consistently effective treatment. Nutritional strategies of avoiding all inflammatory foods, i.e. eliminating sugar and fruit juices entirely and cutting out wheat, dairy eggs and particularly avoiding foods of the nightshade family (potatoes, tomatoes, peppers) has been associated remission of symptoms in sufferers. Carbon dioxide laser surgery is currently considered the last resort for advanced disease. HS is an orphan disease due to sparse research efforts. Suffers may gain insight into how best to control their flare-ups by following blogs written by fellow sufferers. The disease's incidence rate is not well established, but has been estimated as being between 1:24 (4.1%) and 1:600 (0.2%).[medical citation needed]
Hidradenitis suppurativa is a poorly studied disease and its cause remains unknown. Experts disagree over proposed causes.
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:
Post-pubescent individuals are more likely to exhibit HS.
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 are dysfunctional apocrine glands or dysfunctional hair follicles, possibly triggered by a blocked gland, creating inflammation, pain, and a swollen lesion.
HS presents itself in three stages. 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.
Hurley's staging system
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:
Solitary or multiple isolated abscess formation without scarring or sinus tracts. (A few minor sites with rare inflammation; may be mistaken for acne.)
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
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 the following:
Anatomic regions involved (axilla, groin gluteal or other region or infra-mammary region left or right)
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.
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. Possible treatments include the following:
Changes in diet avoiding inflammatory foods, foods high in refined carbohydrates.
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. A few popular antibiotics used to treat hidradenitis suppurativa 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.
Chlorhexidine (Hibiclens) plus an antibiotic soap for cleansing the skin surface. 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 plan for hidradenitis suppurativa.
Topical clindamycin has been shown to have an effect in double-blind placebo controlled studies.
Topical resorcinol is a keratolytic agent that targets the follicular keratin plug and has been shown to have efficacy in several case series studies.
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.
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.
Squamous cell carcinoma: this has been found on rare occasions in chronic hidradenitis suppurativa of the anogenital region. The mean time to the onset of this type of lesion is 10 years or more and the tumors are usually highly aggressive.
In 1839, Velpeau identified and described hidradenitis suppurativa.
In 1854, Verneuil described hidradenitis suppurativa as "Hidrosadénite Phlegmoneuse". This is how HS obtained its alternate name "Verneuil's disease".
In 1922, Schiefferdecker hypothesized a pathogenic link between "Acne inversa" and human apocrine sweat glands.
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". 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". 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.
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".
First description of the hidradenitis suppurativa
Acne triad (hidradenitis suppurativa, perifolliculitis capitis abscendens et suffodiens)
Plewig & Kligman
Acne tetrad (acne triad + pilonidal sinus)
Plewig & Steger
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.
^Stellon AJ, Wakeling M (1989). "Hidradenitis suppurativa associated with use of oral contraceptives". Br Med J298 (6665): 28–9. doi:10.1136/bmj.298.6665.28.
^Gupta AK, Knowles SR, Gupta MA, Jaunkalns R, Shear NH (1995). "Lithium therapy associated with hidradenitis suppurativa: case report and a review of the dermatologic side effects of lithium". J Am Acad Dermatol32 (2 Pt 2): 382–6. doi:10.1016/0190-9622(95)90410-7. PMID7829746.
^Hurley HJ. Axillary hyperhidrosis, apocrine bromhidrosis, hidradenitis suppurativa, and familial benign pemphigus: surgical approach. In: Roenigk RK, Roenigk HH, editors. Dermatologic surgery. Marcel Dekker, New York, 1989, pp. 729–739.
^Sartorius K, Lapins J, Emtestam L, Jemec GB (2003). "Suggestions for uniform outcome variables when reporting treatment effects in hidradenitis suppurativa". Br J Dermatol149 (1): 211–213. doi:10.1046/j.1365-2133.2003.05390.x. PMID12890229.
^Wolkenstein, P; Loundou, A; Barrau, K; Auquier, P; Revuz, J; Quality of Life Group of the French Society of Dermatology (2007). "Quality of life impairment in hidradenitis suppurativa: a study of 61 cases". Journal of the American Academy of Dermatology56 (4): 621–3. doi:10.1016/j.jaad.2006.08.061. PMID17097366.
^Gener G; Canoui-Poitrine F; Revuz JE et al. (2009). "Combination therapy with clindamycin and rifampicin for hidradenitis suppurativa: a series of 116 consecutive patients". Dermatology (Basel)219 (2): 148–54. doi:10.1159/000228334. PMID19590173.
^Scheinfeld N (2013). "Hidradenitis suppurativa: A practical review of possible medical treatments based on over 350 hidradenitis patients". Dermatology Online Journal19 (4): 1. PMID24021361.
^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]". Ann Chir Plast Esthet (in French) 51 (1): 82–6. doi:10.1016/j.anplas.2005.11.002. PMID16488526.