Hidradenitis suppurativa

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File:Hidradenitis.png
A case of hidradenitis suppurativa
File:Hidradenitis Growths.jpg
Hidradenitis suppurativa can take the form of growths on the skin that are extremely painful and debilitating.

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.<ref name="NEJM2012">Template:Cite journal</ref><ref name="GHR2013">{{#invoke:citation/CS1|citation |CitationClass=web }}</ref> These are typically painful and break open, releasing fluid or pus.<ref name="GHR2013" /> The areas most commonly affected are the underarms, under the breasts, perineum, buttocks, and the groin.<ref name="NORD2012">{{#invoke:citation/CS1|citation |CitationClass=web }}</ref> Scar tissue remains after healing.<ref name="NORD2012" /> HS may significantly limit many everyday activities, for instance, walking, hugging, moving, and sitting down. Sitting disability may occur in patients with lesions in the sacral, gluteal, perineal, femoral, groin or genital regions. Prolonged periods of sitting down can also worsen the condition of the skin of these patients.<ref>{{#invoke:citation/CS1|citation |CitationClass=web }}</ref><ref>Template:Cite journal</ref><ref>Template:Cite journal</ref><ref>Template:Cite journal</ref><ref>{{#invoke:citation/CS1|citation |CitationClass=web }}</ref>

The exact cause is usually unclear but believed to involve a combination of genetic and environmental factors.<ref name="GHR2013" /> About a third of people with the disease have an affected family member.<ref name="GHR2013" /> Other risk factors include obesity and smoking.<ref name="GHR2013" /> The condition is not caused by an infection, poor hygiene, or the use of deodorant.<ref name="GHR2013" /><ref name="GARD2017">{{#invoke:citation/CS1|citation |CitationClass=web }}</ref> Instead, it is believed to be caused by hair follicles being obstructed,Template:Refn<ref name="NORD2012" /> with the nearby apocrine sweat glands being strongly implicated in this obstruction.<ref name="NORD2012" /><ref name="2017-NCBI-SeminalSummary">Pathophysiology of hidradenitis suppurativa (Seminal paper, SCMS Journal); NIH, National Library of Medicine, NCBI; 2017 Jun, 36(2):47–54.</ref> The sweat glands may or may not be inflamed.<ref name="NORD2012" /> Diagnosis is based on the symptoms.<ref name="NEJM2012" />

No cure is known,<ref name="GARD2017" /> 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.<ref name="GARD2017" /> Cutting open the lesions to allow them to drain does not result in significant benefit.<ref name="NEJM2012" /> While antibiotics are commonly used, evidence for their use is poor.<ref name="GARD2017" /> Immunosuppressive medication may also be tried.<ref name="NEJM2012" /> In those with more severe disease, laser therapy or surgery to remove the affected skin may be viable.<ref name="NEJM2012" /> Rarely, a skin lesion may develop into skin cancer.<ref name="GHR2013" />

If mild cases of HS are included, then the estimate of its frequency is from 1–4% of the population.<ref name="NEJM2012" /><ref name="GHR2013" /> Women are three times more likely to be diagnosed with it than men.<ref name="NEJM2012" /> Onset is typically in young adulthood and may become less common after 50 years old.<ref name="NEJM2012" /> It was first described between 1833 and 1839 by French anatomist Alfred Velpeau.<ref name="NORD2012" /><ref>Template:Cite book</ref>

Terminology

Although hidradenitis suppurativa is often called acne inversa, it is not a form of acne. Hidradenitis suppurativa lacks the core defining features of acne, such as the presence of closed comedones and increased sebum production.<ref name="Dessinioti2014">Template:Cite journal</ref>

Causes

The exact cause of hidradenitis suppurativa remains unknown,<ref name="2017-NCBI-SeminalSummary" />Template:Refn and there has, in the recent past, been notable disagreement among experts in this regard.<ref name="medline">{{#invoke:citation/CS1|citation |CitationClass=web }}</ref> The condition, however, likely stems from both genetic and environmental causes.<ref name="GHR2013" /> Specifically, an immune-mediated pathology has been proposed,<ref name="2017-NCBI-SeminalSummary" /> although environmental factors have not been ruled out.<ref name="GHR2013" />

Lesions will occur in any body area with hair follicles,<ref name="medline" /> and/or sweat glands.<ref>{{#invoke:citation/CS1|citation |CitationClass=web }}</ref> Areas such as the axilla, groin, and perineal region are more commonly involved. This theory includes most of these potential indicators:<ref name="Schawartzprinciples">Schawartz's Principles of Surgery, 8th edition, self-assessment, and board review, chapter 15, the skin and subcutaneous tissue, question 16</ref>

  • Post-pubescent individuals<ref>{{#invoke:citation/CS1|citation

|CitationClass=web }}</ref>

The historical understanding of the disease suggests dysfunctional apocrine glands<ref name="dermnetnz" /> or dysfunctional hair follicles,<ref>{{#invoke:citation/CS1|citation |CitationClass=web }}</ref> possibly triggered by a blocked gland, which creates inflammation, pain, and a swollen lesion.

Triggering factors

Several triggering factors should be taken into consideration:

Predisposing factors

Some cases have been found to result from mutations in the NCSTN, PSEN1, or PSENEN genes. The genes produce proteins that are all components of a complex called gamma- (γ-) secretase. This complex cuts apart (cleaves) many different proteins, which is a crucial step in several chemical signaling pathways. One of these pathways, known as notch signaling, is essential for the normal maturation and division of hair follicle cells and other types of skin cells. Notch signaling influences normal immune system function. Studies suggest mutations in the NCSTN, PSEN1, or PSENEN genes impair notch signaling in hair follicles. Although little is known about the mechanism, abnormal notch signaling appears to promote the development of nodules and lead to skin inflammation.<ref>{{#invoke:citation/CS1|citation |CitationClass=web }}</ref> In addition, the composition of the intestinal microflora and as a consequence dietary patterns appear to play a role. Although dysbiosis of the cutaneous microbiome apparent in HS is not observed, the concurrent existence of inflammatory gut and skin diseases has led to the hypothesis of a gut-skin axis in which gut microbiota is implicated. Indeed, analysis of bacterial taxa in fecal samples from HS patients supports the possibility of a role for intestinal microbial alterations in this chronic inflammatory skin disease.<ref>Template:Cite journal</ref>

Precocious puberty is more common among children and adolescents with hidradenitis suppurativa (HS) compared to those without the condition, according to a recent case-control study.<ref>{{#invoke:citation/CS1|citation |CitationClass=web }}</ref>Template:Primary source An analysis of the Explorys database revealed that pediatric patients with precocious puberty have double the risk of developing HS, even after adjusting for factors like demographic characteristics and body mass index (BMI).<ref>Template:Cite journal</ref>

Diagnosis

Early diagnosis is essential in avoiding tissue damage. However, HS is often misdiagnosed or diagnosed late due to healthcare professionals not being aware of the condition or people not consulting with a physician.<ref name=":0">Template:Cite journal</ref><ref name=":1">Template:Cite journal</ref> Globally, the diagnosis is delayed more than 7 years in average after symptoms appear. This is much longer than with other skin conditions.<ref>Template:Cite journal</ref>

Stages

File:HS on abdomen.jpg
Hidradenitis suppurativa stage III on abdomen: Skin is red and inflamed, constantly draining a malodorous blood/pus mixture. Pain is severe.
File:HS Hidradenitis Suppurativa.jpg
Hidradenitis suppurativa. This lesion is about 4 inches across.
File:Micrograph of hidradenitis suppurativa.jpg
Micrograph of hidradenitis suppurativa: Sinus tracts [(A), arrow], active inflammation, and rupture [(B), asterisks] of the follicular epithelium with "floating" hair fragments (B), arrowSubsequently, a secondary inflammatory response is induced with an influx of numerous neutrophils [(C), arrows] and granulomatous foreign body reaction with giant cells [(C), asterisks].<ref>Template:Cite journal</ref>

Hidradenitis suppurativa presents itself in three stages.<ref name="dermnetnz">Template:DermNet</ref><ref name="HSUSA">{{#invoke:citation/CS1|citation |CitationClass=web }}</ref> 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.Template:Citation needed

Hurley's staging system

Hurley's staging system was the first classification system proposed and is still in use for the classification of patients with skin 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 to determine appropriate therapy for patients. These three stages are based on Hurley's staging system, which relies on the subjective extent of the diseased tissue. Hurley's three stages of hidradenitis suppurativa are:<ref>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.</ref>

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 restricts 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.)

Sartorius staging system

The Sartorius 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:<ref>Template:Cite journal</ref>

  • Anatomic regions involved (axilla, groin, gluteal, or other region or inframammary region left or right)
  • Number and types of lesions involved (abscesses, nodules, fistulas or sinuses, 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 is present)
  • The presence of normal skin in between lesions (i.e., if all lesions are separated by normal skin)

Points are accumulated in the above categories and added to give 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.<ref>Template:Cite journal</ref>

Treatment

Treatment depends upon the presentation and severity of the disease. Due to the poorly studied nature of the disease, the effectiveness of medications and therapies was unclear.<ref>Template:Cite journal</ref> Clear and sensitive communication from health care professionals, and social and psychological interventions can help manage the emotional impact of the condition and aid necessary lifestyle changes.<ref name=":0" /><ref name=":1" /> In May 2023, the European Commission (EC) approved Cosentyx (secukinumab) for active moderate to severe hidradenitis suppurativa in adults.<ref>Template:Cite journal</ref>

Other possible treatments include the following:

Cryotherapy

Cryotherapy has demonstrated efficacy against the disease, with 88% of persistent lesions resolving in a clinical trial of 23 patients.<ref>Template:Cite journal</ref>

Lifestyle

Warm baths may be tried in those with mild disease.<ref name="GARD2017" /> Weight loss and smoking cessation are recommended.<ref name="NEJM2012" />

Medication

|CitationClass=web }}</ref> are both approved by the FDA for the treatment of HS as of 2023.

  • Topical isotretinoin 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.<ref>Template:Cite journal</ref>
  • Zinc 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.<ref>Template:Cite journal</ref>

Surgery

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 or delayed application of a split-thickness skin graft is an option.<ref name="Schawartzprinciples" /> Another option is covering the defect with a perforator flap. With this technique, the (mostly totally excised) defect is covered with tissue from an area nearby. For example, the axilla with a fully excised defect of 15 × 7 cm can be covered with a thoracodorsal artery perforator flap.Template:Citation needed. A less invasive excision procedure called Skin-Tissue-sparing Excision with Electrosurgical Peeling or "STEEP" has also been developed for treating moderate to severe disease.<ref>Template:Cite journal</ref>

Laser hair removal

Template:Further The 1064 nm wavelength (near-infrared) laser for hair removal may aid in the treatment of HS.<ref>Template:Cite journal</ref> A randomized control study has shown improvement in HS lesions with the use of a Nd:YAG laser.<ref>Template:Cite journal</ref>

Botox injection

A 2022 study reported that administration of Botulinum toxin resulted in either clinical improvement or improved quality of life in 96.8% (n = 30/31) of HS patients. The level of evidence was moderate. It concluded that the treatment was a safe and potentially effective alternative for hidradenitis suppurativa patients resistant to standard-of-care therapies.<ref>Template:Cite journal</ref>

Prognosis

File:HS lesions.jpg
Hidradenitis suppurativa stage III. Open lesions are extremely painful.
File:HS on leg.jpg
Hidradenitis suppurativa stage III. Inflamed lesion.

In stage III disease, as classified by Hurley's staging system, fistulae left undiscovered, undiagnosed, or untreated can rarely lead to the development of squamous cell carcinoma in the anus or other affected areas.<ref>Template:Cite journal</ref><ref>Template:Cite journal</ref> Other stage III chronic sequelae may also include anemia, multi-localized infections, amyloidosis, and arthropathy. Stage III complications have been known to lead to sepsis, but clinical data are still uncertain.

Comorbidities and complications

Endocrine diseases are more common in people who have HS.<ref name="AbuRached2022">Template:Cite journal</ref> Diabetes mellitus may be both a causal factor contributing to the evolution and/or severity of HS and a consequence of inflammation in HS.<ref name="AbuRached2023" /><ref name="AbuRached2024"/> Thyroid disorders are also more common in patients with HS.<ref name="AbuRached2023b">Template:Cite journal</ref>

Impact on mental health

HS is a painful and socially isolating condition that leads to a negative impact on mental health as well. A 2020 meta-analysis found that 21% of people with HS have depression, including major depression and 12% have anxiety, including generalized anxiety disorder and a higher risk of suicide.<ref name="SuppurativeFoundationGuidelines"/><ref>Template:Cite journal</ref> A 2020 study found that people with HS have suicide rates more than double the rates in controls, and also have a higher risk of attempting suicide.<ref name=":2">Template:Cite journal</ref>

Epidemiology

Prevalence

Estimates of the prevalence of HS vary worldwide, and there is no accepted generalization. In the US, the prevalence is estimated to be 0.1% while in Europe it is thought to be 1% or more.<ref name=":2" />

Sex

In North America and Europe, women are three times more likely to have HS. However, in South Korea, men are twice as likely to have HS.<ref name=":2" />

Age

HS is the most prevalent in people in their 40s and 50s.<ref name=":2" />

History

  • From 1833 to 1839, in a series of three publications, Velpeau identified and described a disease now known as hidradenitis suppurativa.<ref>Template:Cite dictionary</ref>
  • In 1854, Aristide Verneuil described hidradenitis suppurativa as {{#invoke:Lang|lang}} ("phlegmonous hidrosadenitis"). This is how HS obtained the name "Verneuil's disease".<ref>Template:Cite journal</ref>
  • In 1922, Paul Schiefferdecker hypothesized a pathogenic link between "acne inversa" and human sweat glands.<ref>Template:Cite bookTemplate:Page needed</ref>
  • In 1956, Pillsbury et al.<ref>Template:Cite book</ref> 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.<ref>Template:Cite journal</ref>
  • 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".<ref>Template:Cite book</ref> 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.<ref name="ReferenceA">Template:Cite book</ref>

A surgeon from Paris, Velpeau, described an unusual inflammatory process with the formation of superficial axillary, submammary, and perianal abscesses, in a series of three publications from 1833 to 1839. One of his colleagues, also located in Paris, named Verneuil, coined the term {{#invoke:Lang|lang}} about 15 years later. This name for the disease reflects the former pathogenetic model of acne inversa, which considered inflammation of sweat glands as the primary cause of hidradenitis suppurativa. In 1922, Paul 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 {{#invoke:Lang|lang}} ("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 hidradenitis suppurativa
Verneuil 1854 lang}}
Pillsbury 1956 lang}}, {{#invoke:Lang|lang}}, {{#invoke:Lang|lang}})
Plewig & Kligman 1975 Acne tetrad (acne triad + pilonidal sinus)
Plewig & Steger 1989 Acne inversa

Other names

Hidradenitis suppurativa has been referred to by multiple names in the literature and various cultures. Some of these are also used to describe different diseases or specific instances of this disease.<ref name="HSUSA" />

  • Acne conglobata – not really a synonym – this is a similar process but in classic acne areas of the chest and back
  • Acne inversa – a proposed new term<ref>Template:Cite journal</ref><ref name="pmid17343599">Template:Cite journal</ref> which has not gained widespread favor.<ref>Template:Cite journal</ref>
  • Apocrine acne – an outdated term based on the disprovenTemplate:Citation needed concept that apocrine glands are primarily involved, though many do have apocrine gland infection
  • Apocrinitis – another outdated term based on the same thesis
  • Fox-den disease – a term not used in medical literature, based on the deep fox den–like sinuses
  • Hidradenitis supportiva – a misspelling
  • Pyodermia fistulans significa – now considered archaic
  • Verneuil's disease – recognizing the surgeon whose name is most often associated with the disorder as a result of his 1854–1865 studies<ref>Template:Cite journal</ref>

Histology

Author Year Major features
Plewig & Steger<ref name="ReferenceA" /> 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<ref>Template:Cite journal</ref> 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<ref name="pmid8977671">Template:Cite journal</ref> 1996 Primary inflammation of the follicular infundibulum. Apocrine sweat glands are secondarily involved.

Society and culture

Experiences of people with HS

HS can have a strong negative impact on people's lives, as well as physical and mental health. People with HS often feel stigmatized 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.<ref name=":0" /><ref name=":1" /> Compared to other skin diseases, HS has one of the highest Dermatology Life Quality Index (DLQI) scores.<ref>Template:Cite journal</ref>

References

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