Hidradenitis suppurativa

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Hidradenitis suppurativa
Classification and external resources
ICD-10L73.2
ICD-9705.83
DiseasesDB5892
eMedicineemerg/259 med/2717 derm/892
MeSHD017497
 
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Hidradenitis suppurativa
Classification and external resources
ICD-10L73.2
ICD-9705.83
DiseasesDB5892
eMedicineemerg/259 med/2717 derm/892
MeSHD017497

Hidradenitis suppurativa (HS) is a skin disease characterized by clusters of chronic abscesses, epidermoid cysts, sebaceous cysts, pilonidal cyst or multilocalised infections that most commonly affects areas bearing apocrine sweat glands, such as the underarms, under the breasts, inner thighs, groin and buttocks.[1] The disease is not contagious.

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.[2] 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.[3] 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.[4]

HS often goes undiagnosed for years due to a delay in seeking treatment and is frequently misdiagnosed.[4] 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%).[5][medical citation needed]

Causes[edit]

Hidradenitis suppurativa is a poorly studied disease and its cause remains unknown. Experts disagree over proposed causes.[6]

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:[7]

The historical understanding of the disease is that there are dysfunctional apocrine glands[11] or dysfunctional hair follicles,[12] 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.

Predisposing factors[edit]

Stages[edit]

HS presents itself in three stages.[4][11] 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[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:[22]

StageCharacteristics
ISolitary or multiple isolated abscess formation without scarring or sinus tracts. (A few minor sites with rare inflammation; may be mistaken for acne.)
IIRecurrent 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.)
IIIDiffuse 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 the following:[23]

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.[24]

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.[25] Possible treatments include the following:

Lifestyle[edit]

Medication[edit]

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.[7]

Laser Hair Removal[edit]

The 1064 nanometer wavelength laser for hair removal aids in the treatment of HS.[36]

Prognosis[edit]

In stage III disease, 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.[37][38] 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]

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".

AuthorYearFindings
Velpeau1839First description of the hidradenitis suppurativa
Verneuil1854"Hidrosadénite phlegmoneuse"
Pillsbury1956Acne triad (hidradenitis suppurativa, perifolliculitis capitis abscendens et suffodiens)
Plewig & Kligman1975Acne tetrad (acne triad + pilonidal sinus)
Plewig & Steger1989Acne 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]

Dermatohistological view of hidradenitis suppurativa[edit]

AuthorYearMajor Features
Plewig & Steger[50]1989Initial 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[55]1990Cysts 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[56]1996Primary inflammation of the follicular infundibulum. Apocrine sweat glands are secondarily involved.

In Popular Culture[edit]

Hidradenitis suppurativa was mentioned on 8 Out of 10 Cats, Season 17 Episode 6.

References[edit]

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External links[edit]