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Hidradenitis suppurativa

Hidradenitis suppurativa (HS), sometimes known as acne inversa or Verneuil's disease, is a long-term dermatological condition characterized by the occurrence of inflamed and swollen lumps.[2][3] These are typically painful and break open, releasing fluid or pus.[3] The areas most commonly affected are the underarms, under the breasts, perineum, buttocks, and the groin.[1] Scar tissue remains after healing.[1] HS may significantly limit many everyday activities, for instance, walking, hugging, moving, and sitting down. Sitting disability may occur in patients with lesions in sacral, gluteal, perineal, femoral, groin or genital regions; and prolonged periods of sitting down can also worsen the condition of the skin of these patients.[5][6][7][8][9]

Hidradenitis suppurativa

Acne inversa, apocrine acne, Verneuil's disease, Velpeau's disease[1]

Multiple inflamed and swollen skin lesions[2]

Childhood and Young adulthood[2]

Long-term[2]

Stage I, II, III[1]

Unknown[3]

Based on symptoms[2]

Warm baths, laser therapy, surgery[2][4]

1–4% of people, when mild cases are included[2][3]

Rare[1]

The exact cause is usually unclear, but believed to involve a combination of genetic and environmental factors.[3] About a third of people with the disease have an affected family member.[3] Other risk factors include obesity and smoking.[3] The condition is not caused by an infection, poor hygiene, or the use of deodorant.[3][4] Instead, it is believed to be caused by hair follicles being obstructed,[10][1] with the nearby apocrine sweat glands being strongly implicated in this obstruction.[1][11] The sweat glands themselves may or may not be inflamed.[1] Diagnosis is based on the symptoms.[2]


No cure is known,[4]though surgical excision with wet-to-dry dressings, proper wound care, and warm baths or showering with a pulse-jet shower may be used in those with mild disease.[4] Cutting open the lesions to allow them to drain does not result in significant benefit.[2] While antibiotics are commonly used, evidence for their use is poor.[4] Immunosuppressive medication may also be tried.[2] In those with more severe disease, laser therapy or surgery to remove the affected skin may be viable.[2] Rarely, a skin lesion may develop into skin cancer.[3]


If mild cases of HS are included, then the estimate of its frequency is from 1–4% of the population.[2][3] Women are three times more likely to be diagnosed with it than men.[2] Onset is typically in young adulthood and may become less common after 50 years old.[2] It was first described between 1833 and 1839 by French anatomist Alfred Velpeau.[1][12]

Terminology[edit]

Although hidradenitis suppurativa is often referred to as acne inversa, it is not a form of acne, and lacks the core defining features of acne such as the presence of closed comedones and increased sebum production.[13]

Post-pubescent individuals

[18]

Blocked hair follicles or blocked

apocrine sweat glands

Excessive sweating

dysfunction

Androgen

that alter cell structure

Genetic disorders

regions involved (axilla, groin gluteal, or other region or inframammary region left or right)

Anatomic

Number and types of involved (abscesses, nodules, fistulas or sinuses, scars, points for lesions of all regions involved)

lesions

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., if all lesions are clearly separated by normal skin)

: taken by mouth, 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. Popular antibiotics also include tetracycline and minocycline.[45] Topical clindamycin has been shown to have an effect in double-blind placebo controlled studies.[46] In a retrospective review and telephone survey, intravenous ertapenem therapy showed clinical improvement with 80.3% of subjects reporting medium to high satisfaction and 90.8% would recommend ertapenem to other patients.[47]

Antibiotics

injections, also known as intralesional steroids, can be particularly useful for localized disease, if the drug can be prevented from escaping via the sinuses.

Corticosteroid

therapy, hormonal therapy with antiandrogenic medications such as spironolactone, flutamide, cyproterone acetate, ethinylestradiol, finasteride, dutasteride, and metformin, have been found to be effective in clinical studies.[48][49][50] However, the quality of available evidence is low and does not presently allow for robust evidence-based recommendations.[48][49]

Antiandrogen

infusion or subcutaneous injection of anti-inflammatory (TNF inhibitors; anti-TNF-alpha) drugs such as infliximab, and etanercept[51] This use of these drugs is not currently Food and Drug Administration (FDA) approved and is somewhat controversial, so may not be covered by insurance.

Intravenous

: Studies have supported that various biologics have a positive effect on HS lesions.[52] Specifically adalimumab at weekly intervals is useful.[53] Adalimumab and Secukinumab[54][55] are both approved by the FDA for the treatment of HS as of 2023.

Biologics

Topical is usually ineffective in people with HS, and is more commonly known as a medication for the treatment of acne vulgaris. Individuals affected by HS who responded to isotretinoin treatment tended to have milder cases of the condition.[56]

isotretinoin

and Nicotinamide, at doses of 90 mg and 30 mg respectively, have shown efficacy against mild to moderate hidradenitis suppurativa in a controlled retrospective clinical trial.[57]

Zinc

and reduced mobility of the lower limbs and axillae due to fibrosis and scarring occur. Severe lymphedema may develop in the lower limbs.

Contractures

Local and systemic (meningitis, bronchitis, pneumonia, etc.), are seen, which may even progress to sepsis.

infections

higher risk for [63]

diabetes mellitus

Interstitial keratitis

Anal, rectal, or urethral [64]

fistulae

Normochromic or hypochromic [65]

anemia

People with HS may be at increased risk for autoimmune disorders including , rheumatoid arthritis, and psoriatic arthritis.[66]

ankylosing spondylitis

has been found on rare occasions in chronic hidradenitis suppurativa of the anogenital region.[67] The mean time to the onset of this type of lesion is 10 years or more and the tumors are usually highly aggressive.

Squamous cell carcinoma

Tumors of the and oral cavity, and liver cancer[68]

lung

and amyloidosis, which can lead to kidney failure and death[69]

Hypoproteinemia

Seronegative and usually asymmetric : pauciarticular arthritis, polyarthritis/polyarthralgia syndrome[70]

arthropathy

Epidemiology[edit]

Prevalence[edit]

Estimates of the prevalence of HS vary worldwide and there is no accepted generalization. In the USA, the prevalence is estimated to be 0.1% while in Europe it is thought to be 1% or more.[72]

From 1833 to 1839, in a series of three publications, Velpeau identified and described a disease now known as hidradenitis suppurativa.

[73]

In 1854, described hidradenitis suppurativa as hidrosadénite Phlegmoneuse. This is how HS obtained its alternate name "Verneuil's disease".[74]

Verneuil

In 1922, Schiefferdecker hypothesized a link between "acne inversa" and human sweat glands.[75]

pathogenic

In 1956, Pillsbury et al. coined the term follicular occlusion triad for the common association of hidradenitis suppurativa, acne conglobata and dissecting cellulitis of the scalp. Modern clinical research still employs Pillsbury's terminology for these conditions' descriptions.[77]

[76]

In 1975, Plewig and Kligman, following Pillsbury's research path, modified the "acne triad", replacing it with the "acne tetrad: acne triad, plus ".[78] Plewig and Kligman's research follows in Pillsbury's footsteps, offering explanations of the symptoms associated with hidradenitis suppurativa.

pilonidal sinus

In 1989, Plewig and Steger's research led them to rename hidradenitis suppurativa, calling it "acne inversa" – which is not still used today in , although some individuals still use this outdated term.[79]

medical terminology

Society and culture[edit]

Experiences of people with HS[edit]

HS can have a strong negative impact on people's lives, as well as physical and mental health. People with HS often feel stigmatised and embarrassed by their condition. Many try to hide the symptoms which can lead to impaired relationships and social isolation. A multidisciplinary approach by healthcare professionals, social support networks and psychological interventions can contribute to a better quality of life.[34][35] Compared to other skin diseases, HS has one of the highest Dermatology Life Quality Index (DLQI) scores.[86]

at American Academy of Dermatology Association

Hidradenitis suppurativa

at British Association of Dermatologists

Hidradenitis suppurativa

Hidradenitis Suppurativa Foundation