Hidradenitis suppurativa

Diseases

Highlights & Basics

Key Highlights

Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease that primarily involves intertriginous areas (i.e., axilla, groin, perineum, and inframammary area).
Treatment is often multidisciplinary. Early referral to an appropriate specialist for discussion of medical and surgical options should be considered.
Medical therapy falls into four main categories: antibiotics, anti-inflammatory medications, hormonal agents, and systemic retinoids. Longer courses of antibiotics are often necessary in order to be effective.
HS is associated with significant morbidity, with development of scarring, chronic pain, lymphedema, and impact on social function.

Hidradenitis suppurativa stage I: discrete inflamed nodules and papules with intervening normal skin and lack of scarring

From R.A. Lee, MD, PhD

Quick Reference

History & Exam
- Key Factors
  - at-risk demographic
  - history of acne vulgaris
  - recurrent disease
  - poor response to previous antibiotic therapy
  - open comedones in intertriginous (axilla, groin, perineum, or inframammary) areas
  - nodules or abscess
  - symmetrical distribution
  - sinus tracts with scarring
- Other Factors
  - premenstrual flare
More information...
Diagnostics Tests
- Other Tests to consider
  - bacterial culture
  - skin biopsy
More information...
Treatment Options
- acute
  - acute abscess
    - antibiotic therapy
    - intralesional corticosteroid
    - incision and drainage
- ongoing
  - mild (Hurley stage I)
    - topical antibacterial or antibiotic
    - antibiotic therapy
    - analgesia
    - lifestyle modifications
More information...

Definition

HS is a chronic inflammatory dermatosis that primarily affects intertriginous areas (axilla, groin, perineum, and inframammary region).[1] [2] While the name and distribution imply that the disease originates in the sweat glands, the pathogenesis is more likely due to involvement of hair follicles, with sweat gland involvement being a secondary event. The disease manifests clinically as inflammatory papules, nodules, and abscesses, and is often associated with scarring.[3] Purulent drainage and sinus tracts can be noted in more advanced disease.

HS is a member of the follicular occlusion tetrad that includes nodulocystic acne, dissecting cellulitis of the scalp, and pilonidal cysts.

Classifications

Hurley classification

The severity of HS can be classified based on the configuration of inflammatory lesions and scars. This method is widely used to stratify disease severity at initial presentation. It is useful to guide treatment choice.[4] [5] [6]

Hurley stage I (mild): presence of abscesses and inflammatory nodules but without scarring.Image
Hurley stage II (moderate): presence of abscesses and inflammatory nodules with scarring. However, inflammatory lesions and scars are separated by areas of intervening normal skin.Image
Hurley stage III (severe): extensive interconnected scars with or without active inflamed lesions.Image

Vignette

Common Vignette 1

A 30-year-old woman presents with recurrent abscesses in the axillae. Previously she has been treated with multiple courses of oral antibiotics with some improvement, but the abscesses recur after cessation of treatment. She notes that flares tend to correspond with her menstrual cycle.

Common Vignette 2

A 28-year-old man presents with multiple draining abscesses in the groin and buttock area. He reports a history of severe acne on his face and back treated with multiple courses of antibiotics and isotretinoin. Lesions on his buttocks and groin have been incised and drained multiple times but invariably recur. He is a smoker. He comes for definitive treatment because his occupation requires him to sit for prolonged periods of time.

Epidemiology

A prevalence estimate of 0.4% has been derived from studies conducted in the US, Europe, and Australia.[7] Studies conducted in the clinical setting suggested a higher pooled prevalence of HS than population-based studies (1.7% vs. 0.3%, respectively).

HS occurs more commonly in females (female to male ratio of approximately 3:1).[8] [9] [10]

Few studies have assessed racial predilection for HS.[11] [12] [13] There is, however, growing evidence to suggest that incidence and prevalence is greater among African-American than white people.[14] [15] [16] [17]

Diagnosis is most common at ages 18 to 29 years.[14] However, delays from disease onset to diagnosis may be as much as 7 years, indicating that onset may occur soon after puberty in some patients.[18]

Etiology

HS is a multifactorial disease with genetic, environmental, lifestyle, and hormonal components. These factors lead to immune activation around the terminal hair follicles and hyperkeratosis.

HS is not an infectious disease, but bacterial propagation in the intertriginous skin, particularly within the occluded hair follicle units, catalyzes immune activation. Inflammation in HS eventually results in extensive pus formation, irreversible tissue destruction, and scar development.[19]

Pathophysiology

HS immunopathogenesis is complex and has characteristics of a neutrophilic dermatosis, with contribution from type 1 helper cells (TH1) and TH17 cells.[19] These activated immunologic pathways lead to insufficient anti-microbial defence, pus formation, and extracellular matrix degradation, contributing to plugging of hair follicles, and recurrent inflammation. Painful nodules and abscesses rupture over time causing sinus tracts and scarring, and progressive tissue destruction.[19]

Patients frequently have metabolic syndrome, type 2 diabetes mellitus, atherosclerosis, spondyloarthritis or spondyloarthropathy, inflammatory bowel disease, and depression.[20] [21] [22] [23] Cardiovascular disease is also increased in people with HS, and contributes to increased mortality rates.[19]

Images

Hidradenitis suppurativa stage I: discrete inflamed nodules and papules with intervening normal skin and lack of scarring
Hidradenitis suppurativa stage II: inflamed nodules and scars with areas of intervening normal skin
Hidradenitis suppurativa stage III: interconnected scars, cysts, comedones, and inflamed nodules
Hidradenitis suppurativa: fluctuant abscess in axilla
Hidradenitis suppurativa: open "double" comedones
Hidradenitis suppurativa: linear scars
Hidradenitis suppurativa: draining sinus tracts

Diagnostic Approach

Diagnosis is usually clinical based on the following presentation:[34]

Typical anatomic location (axillae and inguinal regions; symmetrical lesions suggest systemic disease rather than local infection)
Relapses and chronicity
Typical lesions (deep-seated nodules [boils], comedones, and scarring).

History

For all patients, a full medical and medication history should be taken. Recognized risk factors for HS (e.g., obesity, smoking, positive family history) should be noted.

Patients typically present with painful, firm nodules in the axilla, perineum, and/or groin. Women may report that tenderness and swelling begin about 1 week prior to the onset of menses (premenstrual flare).[35] Patients may experience discomfort or pruritus in the affected region prior to the nodules forming.

Many patients may report previous episodes that may have been unresponsive to antibiotic therapy or that required surgical drainage.[1] Some patients may also report treatment for acne vulgaris.

Comorbidity assessment

Prompt diagnosis and treatment of concomitant disease benefits patient health and quality of life. All patients should be screened for metabolic syndrome, type 2 diabetes, hypertension, Crohn disease, and depression.[36] [37]

Physical exam

Inflammatory nodules and abscesses may be present in the intertriginous areas (axilla, groin, perineum, or inframammary region). Prominent open comedones are often found. Nonintertriginous areas are rarely affected.

Although HS may be unilateral in early disease, most cases will progress to involve both sides of the body. Fibrosis, dermal contractures, disfiguring scars, and sinus tracts may be seen in long-standing disease. ImagesIn persistent peri-anal disease other diagnoses including Crohn disease should be considered.

Investigations

Targeted investigations may be indicated.

Bacterial cultures: can be useful to exclude MRSA furunculosis, and to document change in flora in response to long-term antibiotics.
Skin biopsy: may be performed in select patients to exclude squamous cell carcinoma (SCC) and to differentiate from other inflammatory diseases, including Crohn disease. SCC may be a consequence of long-standing chronic inflammation in advanced HS.[38] [39]
Low zinc levels can manifest as chronic skin erythema in intertriginous areas (acrodermatitis enteropathica); some data suggest that low serum zinc levels are more prevalent in HS than in a healthy population.[40]

View diagnostic guideline references

Risk Factors

strong Factors

Expand All

obesity

- Obesity is a predisposing factor.[21] [24]
- Increasing body mass index in childhood is associated with a greater risk of the development of HS in adulthood.[25]

female sex

- HS occurs more commonly in females (female-to-male ratio of approximately 3:1).[8] [9] [10]

smoking

- Meta-analyses have found that HS is significantly associated with active smoking and a history of smoking.[21] [26] A large retrospective cohort study reported that HS incidence is doubled among tobacco smokers.[27]
- Nicotine increases inflammation and may promote bacterial colonization of intertriginous areas, chemotaxis of inflammatory mediators, and hyperplasia of the infundibular epidermis, thereby contributing to the disease process.[19] [28]

positive family history

- Approximately one third of HS patients report a family history.[29] [30] Patients with a family history may have earlier disease onset and more extensive disease.[31]
- Genetic studies suggest the potential involvement of the γ-secretase pathway in a subset of familial cases.[32] [33]

weak Factors

Expand All

African-American

- Few studies have assessed racial predilection for HS.[11] [12] [13]
- There is, however, growing evidence to suggest that incidence and prevalence is greater among African-American than white people.[14] [15] [16] [17]

History & Exam

Key Factors

Frequency

Expand All

at-risk demographic

common

Approximately one third of HS patients report a family history.[29] [30] HS occurs more commonly in females (female-to-male ratio of approximately 3:1).[8] [9] [10] HS is significantly associated with active smoking and a history of smoking.[21] [26] Obesity is a predisposing factor.[21] [24]

common

history of acne vulgaris

common

Many patients state they have had severe acne as adolescents, and many have had courses of isotretinoin (which effectively manages acne, but not intertriginous lesions).

common

recurrent disease

common

Patients often report multiple trips to the emergency room and treatment with incision and drainage. Bacterial cultures usually do not identify a pathogenic organism.

common

poor response to previous antibiotic therapy

common

Most patients have been given short courses of antibiotics that do not affect the disease course.

common

open comedones in intertriginous (axilla, groin, perineum, or inframammary) areas

common

An important clue to the diagnosis of HS is the presence of multiple open comedones in intertriginous areas.
Axillary and inguinal areas are the most common sites for initial disease.Image

common

nodules or abscess

common

Inflammatory nodules may be present in the intertriginous areas (axilla, groin, perineum, or inframammary region). Fluctuant, tender nodules with purulent drainage are commonly present in patients with active disease.Images

common

symmetrical distribution

common

Although unilateral lesions can present early in the disease course, bilateral involvement is the natural course of the disease.
Persistent unilateral disease warrants consideration of other etiologies.

common

sinus tracts with scarring

common

Linear, cord-like scars with purulent drainage are characteristic of HS. Usually seen in advanced disease.Images

common

Other Factors

Frequency

Expand All

premenstrual flare

common

Many women report that tenderness and swelling begin about 1 week prior to the onset of menses, suggesting a potential role for androgens in HS pathophysiology.[35]

common

Tests

Other Tests to consider

Result

Expand All

bacterial culture

variable; may show methicillin-sensitive Staphylococcus aureus or polymicrobial flora

This is more useful to exclude furunculosis than to make a diagnosis of HS.

variable; may show methicillin-sensitive Staphylococcus aureus or polymicrobial flora

skin biopsy

variable; comedones appear as small, superficial cystic epithelial invaginations filled with keratotic debris; abscesses demonstrate intense acute inflammation in the dermis and sometimes the subcutaneous fat; sinus tracts appear as deep, tunneling epithelial tracts that are often associated with a more chronic inflammatory infiltrate; special stains for bacterial and fungal organisms are usually negative (may see some colonizing bacteria)

Used to exclude squamous cell carcinoma and to differentiate from other inflammatory diseases, including Crohn disease.

Differential Diagnosis

Disease/Condition

Acne vulgaris
Differentiating Signs/Symptoms
Typically occurs on the face, chest, and back. Morphology of lesions can be indistinguishable from HS lesions. However, acne vulgaris rarely results in draining sinus tracts.[2]
Differentiating Tests
No distinguishing tests.
Crohn disease
Differentiating Signs/Symptoms
Fistulas and sinus tracts are usually perianal in distribution. Patients may experience gastrointestinal symptoms, such as bloody diarrhea, abdominal cramps, and lower abdominal pain.[2]
Differentiating Tests
Endoscopy and biopsy will show inflammation with disruption of crypt architecture; noncaseating granulomas may be present.
The presence of granulomas on skin biopsy is suggestive, but not diagnostic, of cutaneous Crohn disease.
Inverse psoriasis
Differentiating Signs/Symptoms
Lesions are usually macerated red patches rather than discrete abscesses or nodules.[2]
Differentiating Tests
Skin biopsy may demonstrate classic findings of psoriasis (regular acanthosis, parakeratosis, and hypogranulosis).
Epidermal inclusion cyst
Differentiating Signs/Symptoms
Typically presents as a solitary lesion, which usually has a visible punctum at the surface. Inflammation is common.[2]
Differentiating Tests
Excisional biopsy is diagnostic and therapeutic.
Furunculosis
Differentiating Signs/Symptoms
Presents as a solitary abscess, often with a collarette of fine scale. More often presents after abrasion of the skin or in immunocompromised patients.[2]
Differentiating Tests
Bacterial culture will demonstrate pathogenic bacteria: for example, Staphylococcus aureus, often MRSA.
Lymphogranuloma venereum
Differentiating Signs/Symptoms
Sexually transmitted disease caused by Chlamydia trachomatis. Typically presents as a suppurative inguinal adenitis with pronounced lymphadenopathy that shows a characteristic groove sign.[2]
Differentiating Tests
Complement fixation test will show a titer >1:64 or a 4-fold rise between acute and convalescent specimens. However, complement fixation with a high titer in the absence of symptoms does not confirm lymphogranuloma venereum, and a low titer does not exclude it.
Squamous cell carcinoma
Differentiating Signs/Symptoms
Usually ulcerated. May be a complication of HS.
Differentiating Tests
Skin biopsy will show evidence of malignancy with foci of keratinization and formation of squamous whorls where the neoplastic cells tightly wrap around each other.

Criteria

Hurley classification[4] [5] [6]

Hurley stage I (mild): presence of abscesses and inflammatory nodules but without scarring.Image
Hurley stage II (moderate): presence of abscesses and inflammatory nodules with scarring. However, inflammatory lesions and scars are separated by areas of intervening normal skin.Image
Hurley stage III (severe): extensive interconnected scars with or without active inflamed lesions.Image

Treatment Approach

The intent of management is to reduce the formation of new inflammatory lesions, sinus tracts, and scarring; to treat existing lesions and reduce associated symptoms (e.g., pain, infection); and to improve associated comorbidities.

Treatment depends on disease severity and impact on quality of life. The Hurley classification scale approximates disease severity at presentation and can be used to stratify patients into groups who receive increasing intensity of treatment. It is useful to guide treatment choice.[4] [36] [41]

Treatment is often multidisciplinary.[1] [42] Early referral to a dermatologist is recommended if patients don't respond to one to two courses of long-term (3 months) antibiotics in the community. Medical therapy includes use of topical and oral antibiotics, retinoids, and in moderate to severe disease, biologic agents. Discussion of surgical options should also be considered.

Acute symptoms

In the acute setting, oral antibiotics should be initiated. Guidelines recommend a 12-week course of a tetracycline antibiotic.[42] [43] [44]

In severe HS presenting with a disease flare, rescue therapy in the form of a 6-week course of intravenous ertapenem, followed by a 6-week course of consolidation treatment with moxifloxacin plus metronidazole plus rifampin, may be considered.[43] [44] [45] [46]

If the patient is well, intralesional corticosteroids may provide relief from pain and inflammation, in combination with oral antibiotics if required.[42] [43] [44] [47]

If the patient is unwell or disease does not improve with antibiotics and/or intralesional corticosteroids, incision and drainage may be considered.[44] Incision and drainage is a supplemental measure; it should not be considered as the sole treatment because recurrence is very common.

Ongoing management: mild disease (Hurley Stage I)

Stage I is defined as the presence of localised disease with inflammatory papules, pustules, nodules, and abscesses but without sinus tracts or scarring.[4]Image

Topical therapies

It is recommended that all patients use an antimicrobial wash, but there is no strong evidence for specific agents; use of chlorhexidine, benzoyl peroxide, and zinc pyrithione is supported by expert opinion. Concomitant use of an antimicrobial wash may be associated with lower rates of antibiotic resistance in HS lesions.[43] [48]

Topical antibiotics are useful treatments in mild HS.[41] Topical clindamycin has been shown to be effective in a clinical trial setting.[43] [49] Topical metronidazole is another option. Topical therapy should be continued for a minimum of 8 weeks before evaluation of efficacy.

Antibiotic therapy

Systemic antibiotics are widely used and recommended in multiple HS treatment guidelines.[42] [43] [44] Oral tetracyclines are recommended first-line for mild disease. They can attenuate neutrophil activity and reduce pain and inflammation. A 12-week treatment course is recommended before evaluation of efficacy.[42] [43] [44]

Analgesia

The degree of pain usually correlates with the degree of inflammation. Thus, treatments directed at inflammation are often effective at alleviating pain. Nonsteroidal anti-inflammatory drugs should be used as required before other pain medications such as acetaminophen.

Lifestyle modifications

A high proportion of patients with hidradentitis suppurativa are active smokers or have a history of smoking, and are obese. Obesity is an independent risk factor for development of the disease and contributes to HS disease severity.[21] [24] All patients should be advised to stop smoking, to lose weight if obese, and to be evaluated for cardiovascular disease.[44] [50]

Ongoing management: moderate disease (Hurley Stage II)

Stage II is defined as the presence of inflammatory papules and nodules, ≥1 recurring, widely separated abscesses with sinus tracts and scarring.[4] [41]Image

Antibiotic therapy

Tetracyclines are recommended for 12 weeks.[42] [43]

Clindamycin plus rifampin is an effective combination and should be continued for 10 to 12 weeks.[42] [43] [44] However, patients often relapse within 4 to 5 months of stopping therapy.[43] [44] [51]

Diarrhea may reduce tolerability of clindamycin plus rifampin in some patients.[42] [43] [44] Patients who are ages ≥50 years or ever-smokers are less likely to tolerate this combination.[36] [52] [53]

Rifampin induces the cytochrome P450 system; check for potential drug interactions with existing medication including the oral contraceptive pill. Clindamycin plus rifampin may also select for rifampin-resistant strains of Mycobacterium tuberculosis; tuberculosis screening or avoiding this regimen may be indicated in high-risk populations.[54]

Triple antibiotic therapy with moxifloxacin plus metronidazole plus rifampin is a second-line option for moderate HS.[43]

Dapsone

Dapsone is reserved for patients with moderate disease who have failed multiple courses of combination antibiotics.[42] [43] Evidence is limited; in one published case series, 25% patients experienced clinically significant improvement but with rapid disease recurrence upon cessation.[55]

Antiandrogens

North American guidelines suggest that hormonal agents, including spironolactone, may be considered in women with clear premenstrual flares (while recognising that recommendations regarding hormonal therapies are based on limited evidence).[43]

Use of spironolactone should be limited to women who are practicing adequate birth control.

UK guidelines conclude that there is insufficient evidence to recommend antiandrogens for the treatment of HS.[42] [56]

Oral retinoids

Patients with concomitant acne vulgaris may consider using oral isotretinoin.[42] [43] [57] Oral isotretinoin should be continued for at least 6 months.

Acitretin has demonstrated moderate efficacy in HS.[43] [58] Treatment duration in excess of 6 months has been reported.[59]

These medications are teratogenic and should be avoided in women of child-bearing potential.

Surgical management

Wide excision with wide and deep margins is the standard of care for surgical therapy. Because of the size of the excision and its corresponding repair, and the availability of specific lasers, referral to plastic surgery and/or a dermatologic surgeon is advisable.[60] [61] [62] [63]

Local excision is possible for smaller, quiescent lesions where the clinical margins can be clearly defined.

Adjunctive therapies and self-management

Topical therapies, analgesia, and lifestyle modifications may be considered (as for patients with mild disease).

Ongoing management: severe disease (Hurley Stage III)

Stage III disease is defined as multiple abscesses and interconnected sinus tracts and scars.[4]Image

The strategies for stage III disease overlap with those for stage I and II disease. In practice, patients in this group will have previously used oral tetracyclines, and they may be used again in between other measures to maintain disease control in stage III disease. However, once disease reaches stage III more aggressive treatment is usually required.

Antibiotic therapy

In selected stage III patients with severe HS presenting with a disease flare (usually after failure of a tetracycline [for 12 weeks] or clindamycin plus rifampin), a 6-week course of intravenous ertapenem, followed by a 6-week course of consolidation treatment with moxifloxacin plus metronidazole plus rifampin, may be considered.[43] [44] [45] [46]

Biologic agents

Adalimumab has demonstrated efficacy in phase 3 trials of HS.[64] It is the only approved biologic for HS, and is therefore considered the ﬁrst-choice biologic agent in moderate/severe HS refractory to conventional treatments.[42] [43] [44]

Inﬂiximab may be considered as a second-line biologic agent, and is used off-label for HS.[42] [43] [44]

Other biologic agents may be considered if adalimumab or infliximab fail or are contraindicated, but their use is off-label and should be under specialist guidance.[43]

Therapy with biologics is continued for at least 12 weeks and the efficacy of treatment assessed at this time.

Surgical management

Local excision is possible for smaller, quiescent lesions where the clinical margins can be clearly defined.

Adjunctive therapies and self-management

Topical therapies, analgesia, and lifestyle modifications may be considered (as for patients with mild disease).

Women with clear premenstrual flares should consider using antiandrogen medications such as spironolactone.[43] Oral retinoids may be considered in patients with concomitant acne vulgaris.[42] [43]

View treatment guideline references

Treatment Options

acute
Expand All
- acute abscess
  - - 1st
      antibiotic therapy
      Primary Options
      tetracycline
      500 mg orally twice daily
      doxycycline
      100 mg orally twice daily
      minocycline
      100 mg orally twice daily
      Secondary Options
      ertapenem
      1 g intravenously every 24 hours
      and
      moxifloxacin
      400 mg orally once daily
      and
      metronidazole
      500 mg orally three times daily
      and
      rifampin
      300 mg orally twice daily
      Comments
      Guidelines recommend a 12-week course of a tetracycline antibiotic.[42] [43] [44]
      In severe HS presenting with a disease flare, rescue therapy in the form of a 6-week course of intravenous ertapenem, followed by a 6-week course of consolidation treatment with moxifloxacin plus metronidazole plus rifampin, may be considered.[43] [44] [45] [46]
    - plus
      intralesional corticosteroid
      Primary Options
      triamcinolone acetonide
      consult specialist for guidance on dose
      Comments
      If the patient is well, intralesional corticosteroids may provide relief from pain and inflammation, in combination with oral antibiotics if required.[42] [43] [44] [47]
    - adjunct
      incision and drainage
      Comments
      If the patient is unwell or disease does not improve with antibiotics and/or intralesional corticosteroids, incision and drainage may be considered.[44] Incision and drainage is a supplemental measure; it should not be considered as the sole treatment because recurrence is very common.
ongoing
Expand All
- mild (Hurley stage I)
  - - 1st
      topical antibacterial or antibiotic
      Primary Options
      clindamycin topical
      (1%) apply to the affected area(s) twice daily
      metronidazole topical
      (0.75%) apply to the affected area(s) twice daily; (1%) apply to the affected area(s) once daily
      Comments
      Stage I is defined as the presence of localised disease with inflammatory papules, pustules, nodules, and abscesses but without sinus tracts or scarring.[4]Image
      It is recommended that patients use an antimicrobial wash, but there is no strong evidence for specific agents; use of chlorhexidine, benzoyl peroxide, and zinc pyrithione is supported by expert opinion. Concomitant use of an antimicrobial wash may be associated with lower rates of antibiotic resistance in HS lesions.[43] [48]
      Topical antibiotics are useful treatments in mild HS.[41] Topical clindamycin has been shown to be effective in a clinical trial setting.[43] [49] Topical metronidazole is another option. Topical therapy should be continued for a minimum of 8 weeks before evaluation of efficacy.
    - adjunct
      antibiotic therapy
      Primary Options
      tetracycline
      500 mg orally twice daily
      doxycycline
      100 mg orally twice daily
      minocycline
      100 mg orally twice daily
      Comments
      Oral tetracyclines are recommended first-line for mild disease. They can attenuate neutrophil activity and reduce pain and inflammation. A treatment course of 12-weeks is recommended before evaluation of efficacy.[42] [43] [44]
    - adjunct
      analgesia
      Primary Options
      ibuprofen
      400 mg orally every 4-6 hours when required, maximum 2400 mg/day
      Secondary Options
      acetaminophen
      325-1000 mg orally every 4-6 hours when required, maximum 4000 mg/day
      Comments
      The degree of pain usually correlates with the degree of inflammation. Thus, treatments directed at inflammation are often effective at alleviating pain. Nonsteroidal anti-inflammatory drugs should be used as required before other pain medications such as acetaminophen.
    - adjunct
      lifestyle modifications
      Comments
      A high proportion of patients with hidradentitis suppurativa are active smokers or have a history of smoking, and are obese. Obesity is an independent risk factor for development of the disease and contributes to HS disease severity.[21] [24] All patients should be advised to stop smoking, to lose weight if obese, and to be evaluated for cardiovascular disease.[44] [50]
- moderate (Hurley stage II)
  - - 1st
      antibiotic therapy
      Primary Options
      tetracycline
      500 mg orally twice daily
      doxycycline
      100 mg orally twice daily
      minocycline
      100 mg orally twice daily
      clindamycin
      300 mg orally twice daily
      and
      rifampin
      300 mg orally twice daily
      Secondary Options
      moxifloxacin
      400 mg orally once daily
      and
      metronidazole
      500 mg orally three times daily
      and
      rifampin
      300 mg orally twice daily
      Comments
      Stage II is defined as the presence of inflammatory papules and nodules, ≥1 recurring, widely separated abscesses with sinus tracts and scarring.[4] [41]Image
      Tetracyclines are recommended for 12 weeks.[42] [43]
      Clindamycin plus rifampin is an effective combination and should be continued for 10 to 12 weeks.[42] [43] [44] However, patients often relapse within 4 to 5 months of stopping therapy.[43] [44] [51]
      Diarrhea may reduce tolerability of clindamycin plus rifampin in some patients.[42] [43] [44] Patients who are ages ≥50 years or ever-smokers are less likely to tolerate this combination.[36] [52] [53]
      Rifampin induces the cytochrome P450 system; check for potential drug interactions with existing medication including the oral contraceptive pill. Clindamycin plus rifampin may also select for rifampin-resistant strains of Mycobacterium tuberculosis; tuberculosis screening or avoiding this regimen may be indicated in high-risk populations.[54]
      Triple antibiotic therapy with moxifloxacin plus metronidazole plus rifampin is a second-line option for moderate HS.[43]
    - plus
      topical antibiotic or antibacterial
      Primary Options
      clindamycin topical
      (1%) apply to the affected area(s) twice daily
      metronidazole topical
      (0.75%) apply to the affected area(s) twice daily; (1%) apply to the affected area(s) once daily
      Comments
      It is recommended that patients use an antimicrobial wash, but there is no strong evidence for specific agents; use of chlorhexidine, benzoyl peroxide, and zinc pyrithione is supported by expert opinion. Concomitant use of an antimicrobial wash may be associated with lower rates of antibiotic resistance in HS lesions.[43] [48] These products are available over the counter.
      Topical antibiotics are useful treatments in mild HS.[41] Topical clindamycin has been shown to be effective in a clinical trial setting.[43] [49] Topical metronidazole is another option. Topical therapy should be continued for a minimum of 8 weeks before evaluation of efficacy.
    - adjunct
      analgesia
      Primary Options
      ibuprofen
      400 mg orally every 4-6 hours when required, maximum 2400 mg/day
      Secondary Options
      acetaminophen
      325-1000 mg orally every 4-6 hours when required, maximum 4000 mg/day
      Comments
      The degree of pain usually correlates with the degree of inflammation. Thus, treatments directed at inflammation are often effective at alleviating pain. Nonsteroidal anti-inflammatory drugs should be used as required before other pain medications such as acetaminophen.
    - adjunct
      lifestyle modifications
      Comments
      A high proportion of patients with hidradentitis suppurativa are active smokers or have a history of smoking, and are obese. Obesity is an independent risk factor for development of the disease and contributes to HS disease severity.[21] [24] All patients should be advised to stop smoking, to lose weight if obese, and to be evaluated for cardiovascular disease.[44] [50]
    - 2nd
      dapsone
      Primary Options
      dapsone
      50-200 mg orally once daily
      Comments
      Dapsone is reserved for patients with moderate disease who have failed multiple courses of combination antibiotics.[42] [43] Evidence is limited; in one published case series, 25% patients experienced clinically significant improvement but with rapid disease recurrence upon cessation.[55]
    - plus
      topical antibiotic or antibacterial
      Primary Options
      clindamycin topical
      (1%) apply to the affected area(s) twice daily
      metronidazole topical
      (0.75%) apply to the affected area(s) twice daily; (1%) apply to the affected area(s) once daily
      Comments
      It is recommended that patients use an antimicrobial wash, but there is no strong evidence for specific agents; use of chlorhexidine, benzoyl peroxide, and zinc pyrithione is supported by expert opinion. Concomitant use of an antimicrobial wash may be associated with lower rates of antibiotic resistance in HS lesions.[43] [48] These products are available over the counter.
      Topical antibiotics are useful treatments in mild HS.[41] Topical clindamycin has been shown to be effective in a clinical trial setting.[43] [49] Topical metronidazole is another option. Topical therapy should be continued for a minimum of 8 weeks before evaluation of efficacy.
    - adjunct
      analgesia
      Primary Options
      ibuprofen
      400 mg orally every 4-6 hours when required, maximum 2400 mg/day
      Secondary Options
      acetaminophen
      325-1000 mg orally every 4-6 hours when required, maximum 4000 mg/day
      Comments
      The degree of pain usually correlates with the degree of inflammation. Thus, treatments directed at inflammation are often effective at alleviating pain. Nonsteroidal anti-inflammatory drugs should be used as required before other pain medications such as acetaminophen.
    - adjunct
      lifestyle modifications
      Comments
      A high proportion of patients with hidradentitis suppurativa are active smokers or have a history of smoking, and are obese. Obesity is an independent risk factor for development of the disease and contributes to HS disease severity.[21] [24] All patients should be advised to stop smoking, to lose weight if obese, and to be evaluated for cardiovascular disease.[44] [50]
  - with premenstrual flare
    - adjunct
      spironolactone
      Primary Options
      spironolactone
      100-150 mg orally once daily
      Comments
      North American guidelines suggest that hormonal agents, including spironolactone, may be considered in women with clear premenstrual flares (while recognising that recommendations regarding hormonal therapies are based on limited evidence).[43]
      Use of spironolactone should be limited to women who are practicing adequate birth control.
      Treatment should be continued for at least 8 weeks.
  - with concomitant acne vulgaris
    - adjunct
      oral retinoid
      Primary Options
      isotretinoin
      0.5 to 1 mg/kg/day orally given in 2 divided doses
      Secondary Options
      acitretin
      25-50 mg orally once daily
      Comments
      Patients with concomitant acne vulgaris may consider using oral isotretinoin.[42] [43] [57]
      Before treatment, patients require counseling about the potential adverse effects. Severe headaches, decreased night vision, or signs of adverse psychiatric events are indications for prompt discontinuation. Complete blood count, lipid panel, and liver function tests are monitored regularly.
      Isotretinoin is teratogenic; therefore, women undergo pregnancy testing before starting isotretinoin and monthly while taking the drug. In the US, isotretinoin can be prescribed only through the iPledge system.iPledge system (for isotretinoin prescribing) Oral isotretinoin should be continued for at least 6 months.
      Acitretin has demonstrated moderate efficacy in HS.[43] [58] Treatment duration in excess of 6 months has been reported.[59] Acitretin is teratogenic and should be avoided in women of child-bearing potential.
  - with scarring
    - adjunct
      surgical repair
      Comments
      Wide excision with wide and deep margins is the standard of care for surgical therapy. Because of the size of the excision and its corresponding repair, and the availability of specific lasers, referral to plastic surgery and/or a dermatologic surgeon is advisable.[60] [61] [62] [63]
      Local excision is possible for smaller, quiescent lesions where the clinical margins can be clearly defined.
      Destructive methods, such as cryotherapy, are generally not recommended. Selective use of deroofing of individual epithelialized sinus tracts can be effective in treating specific recurrent lesions.
- severe (Hurley stage III)
  - - 1st
      antibiotic therapy
      Primary Options
      tetracycline
      500 mg orally twice daily
      doxycycline
      100 mg orally twice daily
      minocycline
      100 mg orally twice daily
      clindamycin
      300 mg orally twice daily
      and
      rifampin
      300 mg orally twice daily
      Secondary Options
      ertapenem
      1 g intravenously every 24 hours
      and
      moxifloxacin
      400 mg orally once daily
      and
      metronidazole
      500 mg orally three times daily
      and
      rifampin
      300 mg orally twice daily
      Comments
      Stage III disease is defined as multiple abscesses and interconnected sinus tracts and scars.[4]Image
      In practice, patients in this group will have previously used oral tetracyclines, and they may be used again in between other measures to maintain disease control in stage III disease. However, once disease reaches stage III more aggressive treatment is usually required.
      In selected stage III patients with severe HS presenting with a disease flare (usually after failure of a tetracycline [for 12 weeks] or clindamycin plus rifampin), a 6-week course of intravenous ertapenem, followed by a 6-week course of consolidation treatment with moxifloxacin plus metronidazole plus rifampin, may be considered.[43] [44] [45] [46]
    - plus
      topical antibiotic or antibacterial
      Primary Options
      clindamycin topical
      (1%) apply to the affected area(s) twice daily
      metronidazole topical
      (0.75%) apply to the affected area(s) twice daily; (1%) apply to the affected area(s) once daily
      Comments
      It is recommended that all patients use an antimicrobial wash, but there is no strong evidence for specific agents; use of chlorhexidine, benzoyl peroxide, and zinc pyrithione is supported by expert opinion. Concomitant use of an antimicrobial wash may be associated with lower rates of antibiotic resistance in HS lesions.[43] [48] These products are available over the counter.
      Topical antibiotics are useful treatments in mild HS.[41] Topical clindamycin has been shown to be effective in a clinical trial setting.[43] [49] Topical metronidazole is another option. Topical therapy should be continued for a minimum of 8 weeks before evaluation of efficacy.
    - adjunct
      analgesia
      Primary Options
      ibuprofen
      400 mg orally every 4-6 hours when required, maximum 2400 mg/day
      Secondary Options
      acetaminophen
      325-1000 mg orally every 4-6 hours when required, maximum 4000 mg/day
      Comments
      The degree of pain usually correlates with the degree of inflammation. Thus, treatments directed at inflammation are often effective at alleviating pain. Nonsteroidal anti-inflammatory drugs should be used as required before other pain medications such as acetaminophen.
    - adjunct
      tumor necrosis factor (TNF)-alpha inhibitor
      Primary Options
      adalimumab
      160 mg subcutaneously on day 1, followed by 80 mg on day 15, then 40 mg once weekly or 80 mg every 2 weeks starting on day 29
      Secondary Options
      infliximab
      consult specialist for guidance on dose
      Comments
      Adalimumab has demonstrated efficacy in phase 3 trials of HS.[64] It is the only approved biologic for HS, and is therefore considered the ﬁrst-choice biologic agent in moderate/severe HS refractory to conventional treatments.[42] [43] [44]
      Inﬂiximab may be considered as a second-line biologic agent, and is used off-label for HS.[42] [43] [44]
      Other biologic agents may be considered if adalimumab or infliximab fail or are contraindicated, but their use is off-label and should be under specialist guidance.[43]
      Therapy with biologics is continued for at least 12 weeks and the efficacy of treatment assessed at this time.
    - adjunct
      lifestyle modifications
      Comments
      A high proportion of patients with hidradentitis suppurativa are active smokers or have a history of smoking, and are obese. Obesity is an independent risk factor for development of the disease and contributes to HS disease severity.[21] [24] All patients should be advised to stop smoking, to lose weight if obese, and to be evaluated for cardiovascular disease.[44] [50]
  - with premenstrual flare
    - adjunct
      spironolactone
      Primary Options
      spironolactone
      100-150 mg orally once daily
      Comments
      North American guidelines suggest that hormonal agents, including spironolactone, may be considered in women with clear premenstrual flares (while recognising that recommendations regarding hormonal therapies are based on limited evidence).[43]
      Use of spironolactone should be limited to women who are practicing adequate birth control.
      Treatment should be continued for at least 8 weeks.
  - with concomitant acne vulgaris
    - adjunct
      oral retinoid
      Primary Options
      isotretinoin
      0.5 to 1 mg/kg/day orally given in 2 divided doses
      Secondary Options
      acitretin
      25-50 mg orally once daily
      Comments
      Patients with concomitant acne vulgaris may consider using oral isotretinoin.[42] [43] [57]
      Before treatment, patients require counseling about the potential adverse effects. Severe headaches, decreased night vision, or signs of adverse psychiatric events are indications for prompt discontinuation. Complete blood count, lipid panel, and liver function tests are monitored regularly.
      Isotretinoin is teratogenic; therefore, women undergo pregnancy testing before starting isotretinoin and monthly while taking the drug. In the US, isotretinoin can be prescribed only through the iPledge system.iPledge system (for isotretinoin prescribing) Oral isotretinoin should be continued for at least 6 months.
      Acitretin has demonstrated moderate efficacy in HS.[43] [58] Treatment duration in excess of 6 months has been reported.[59] Acitretin is teratogenic and should be avoided in women of child-bearing potential.
  - with scarring
    - adjunct
      surgical repair
      Comments
      Wide excision with wide and deep margins is the standard of care for surgical therapy. Because of the size of the excision and its corresponding repair, and the availability of specific lasers, referral to plastic surgery and/or a dermatologic surgeon is advisable.[60] [61] [62] [63]
      Local excision is possible for smaller, quiescent lesions where the clinical margins can be clearly defined.
      Destructive methods, such as cryotherapy, are generally not recommended. Selective use of deroofing of individual epithelialized sinus tracts can be effective in treating specific recurrent lesions.

Emerging Tx

Anakinra

Results from a small double-blind, randomized, placebo-controlled trial suggest that anakinra, an interleukin (IL)-1 receptor antagonist, may prolong time to a new HS exacerbation.[65]

Topical resorcinol

Uncontrolled studies suggest that topical resorcinol 15%, a keratolytic and antiseptic, may benefit patients with mild to moderate HS.[66] [67] Adverse effects may include skin irritation, pigment change, and desquamation.[66] Topical resorcinol may need to be compounded by a pharmacy.

Ustekinumab

Ustekinumab, an IL-12/23 inhibitor, was found to be moderately effective (response rate approximately 50%) in an open-label study of patients with HS.[68] A subsequent literature review reported that, in most cases, HS begins to improve 3 to 5 months after initiation of ustekinumab.[69]

Secukinumab

A human monoclonal antibody that inhibits IL-17A. A systematic review of available treatment outcomes suggests that 57.1% (n=60/105) of HS patients treated with secukinumab were responders (mean response period of 16 weeks).[70] Phase 3 studies are recruiting or ongoing.[71]

Finasteride

North American guidelines suggest that hormonal agents, including finasteride, may be considered in women with clear premenstrual flares (while recognizing that recommendations regarding hormonal therapies are based on limited evidence).[43] A retrospective chart review found that androgen blockade therapy with finasteride is safe and effective among female patients with HS with a contraindication or intolerance to spironolactone.[72]

Laser or light-based therapy

One Cochrane review reported on three small randomized controlled trials (RCTs) of laser or light treatment for HS, concluding that their low quality made it difficult to make treatment recommendations.[73] A subsequent systematic review concluded that there is a need for large RCTs.[74]

Follow-Up Overview

Prognosis

The natural course of mild disease can be benign and controlled with medical intervention. Limited moderate disease requires more aggressive medical and possible surgical therapy for adequate control. Poorly controlled moderate disease can progress to more severe disease. Complete remission from symptoms is unlikely with medical therapy alone. Care should be taken to evaluate for evidence of systemic infection. Consultation with plastic surgery is advisable for moderate to severe disease. Long-standing severe disease can result in lymphedema and development of squamous cell carcinoma. The impact on patients' quality of life can be profound.[75] [76]

Monitoring

Frequency of monitoring depends on the severity of the disease, as well as the specific medications used. Careful physical examination is important, particularly if inflammation is active, and should include assessment of signs of infection and possible transformation into squamous cell carcinoma.

Routine assessment and intervention for the treatment of depression and anxiety is recommended.[75] [76]

Complications

High Likelihood

Timeframe

Expand All

scarring

variable

Severe HS may leave residual scars in previously involved areas.Image

variable

localized infection/cellulitis

variable

Localized infection is common. Development of drug-resistant organisms is likely with long-term antibiotic use. Serial bacterial cultures are useful, particularly when there is an unexpected flare of the disease.

variable

Medium Likelihood

Timeframe

Expand All

sepsis

variable

Systemic infection is uncommon, but the risk is increased particularly in patients on systemic immunosuppressive medications.[77]

variable

Low Likelihood

Timeframe

Expand All

squamous cell carcinoma

long term

Chronic inflammation is a risk factor for developing squamous cell carcinoma (SCC). Patients with long-standing severe HS are more likely to develop SCC, particularly in the perianal and inguinal areas. This occurs more often in men. The average duration of symptomatic HS in patients who develop SCC is 25 years. Careful examination of draining and ulcerated lesions is critical for early identification and treatment.[78] [79]

long term

lymphedema

long term

Chronic inflammation can lead to scarring of the lymphatics and lead to poor drainage of local interstitial fluid, leading to lymphedema. Aggressive treatment is important to avoid this outcome. Once lymphedema is present, treatment is difficult. Referral to physical therapy for possible compression therapy may be considered.[78]

long term

Citations

Key Articles

Kim WB, Sibbald RG, Hu H, et al. Clinical features and patient outcomes of hidradenitis suppurativa: a cross-sectional retrospective study. J Cutan Med Surg. 2016;20:52-57.[Abstract][Full Text]
Martorell A, García-Martínez FJ, Jiménez-Gallo D, et al. An update on hidradenitis suppurativa (part I): epidemiology, clinical aspects, and definition of disease severity. Actas Dermosifiliogr. 2015;106:703-715.[Abstract][Full Text]
Sabat R, Jemec GBE, Matusiak Ł, et al. Hidradenitis suppurativa. Nat Rev Dis Primers. 2020 Mar 12;6(1):18.[Abstract]
Martorell A, García FJ, Jiménez-Gallo D, et al. Update on hidradenitis suppurativa (part II): treatment. Actas Dermosifiliogr. 2015;106:716-724.[Abstract][Full Text]
Kimball AB, Okun MM, Williams DA, et al. Two phase 3 trials of adalimumab for hidradenitis suppurativa. N Engl J Med. 2016;375:422-34.[Abstract][Full Text]
Ingram JR, Woo PN, Chua SL, et al. Interventions for hidradenitis suppurativa. Cochrane Database Syst Rev. 2015;(10):CD010081.[Abstract][Full Text]

Other Online Resources

iPledge system (for isotretinoin prescribing)
Hope for HS

Referenced Articles

1. Jemec GB. Clinical practice. Hidradenitis suppurativa. N Engl J Med. 2012;366:158-164.[Abstract]
2. Lee RA, Yoon A, Kist J. Hidradenitis suppurativa: an update. Adv Dermatol. 2007;23:289-306.[Abstract]
3. Jemec GB. Hidradenitis suppurativa. J Cutan Med Surg. 2003;7:47-56.[Abstract]
4. Hurley H. Axillary hyperhidrosis, apocrine bromhidrosis, hidradenitis suppurativa, and familial benign pemphigus. In: Roenigk RH, Roenigk HH Jr, eds. Dermatologic surgery: principles and practice. New York, NY: Marcel Dekker; 1989:729-739.
5. Kim WB, Sibbald RG, Hu H, et al. Clinical features and patient outcomes of hidradenitis suppurativa: a cross-sectional retrospective study. J Cutan Med Surg. 2016;20:52-57.[Abstract][Full Text]
6. Martorell A, García-Martínez FJ, Jiménez-Gallo D, et al. An update on hidradenitis suppurativa (part I): epidemiology, clinical aspects, and definition of disease severity. Actas Dermosifiliogr. 2015;106:703-715.[Abstract][Full Text]
7. Jfri A, Nassim D, O'Brien E, et al. Prevalence of hidradenitis suppurativa: a systematic review and meta-regression analysis. JAMA Dermatol. 2021 Aug 1;157(8):924-31.[Abstract]
8. Ingram JR, Jenkins-Jones S, Knipe DW, et al. Population-based clinical practice research datalink study using algorithm modelling to identify the true burden of hidradenitis suppurativa. Br J Dermatol. 2018 Apr;178(4):917-24.[Abstract][Full Text]
9. Garg A, Kirby JS, Lavian J, et al. Sex- and age-adjusted population analysis of prevalence estimates for hidradenitis suppurativa in the United States. JAMA Dermatol. 2017 Aug 1;153(8):760-64.[Abstract][Full Text]
10. Hallock KK, Mizerak MR, Dempsey A, et al. Differences between children and adults with hidradenitis suppurativa. JAMA Dermatol. 2021 Sep 1;157(9):1095-1101.[Abstract]
11. Sachdeva M, Shah M, Alavi A. Race-specific prevalence of hidradenitis suppurativa. J Cutan Med Surg. 2021 Mar-Apr;25(2):177-87.[Abstract]
12. Price KN, Hsiao JL, Shi VY. Race and ethnicity gaps in global hidradenitis suppurativa clinical trials. Dermatology. 2021;237(1):97-102.[Abstract][Full Text]
13. Zouboulis CC, Goyal M, Byrd AS. Hidradenitis suppurativa in skin of colour. Exp Dermatol. 2021 Jun;30(suppl 1):27-30.[Abstract][Full Text]
14. Garg A, Lavian J, Lin G, et al. Incidence of hidradenitis suppurativa in the United States: a sex- and age-adjusted population analysis. J Am Acad Dermatol. 2017 Jul;77(1):118-22.[Abstract]
15. Vlassova N, Kuhn D, Okoye GA. Hidradenitis suppurativa disproportionately affects African Americans: a single-center retrospective analysis. Acta Derm Venereol. 2015 Nov;95(8):990-1.[Abstract][Full Text]
16. Garg A, Wertenteil S, Baltz R, et al. Prevalence estimates for hidradenitis suppurativa among children and adolescents in the United States: a gender- and age-adjusted population analysis. J Invest Dermatol. 2018 Oct;138(10):2152-6.[Abstract][Full Text]
17. Vaidya T, Vangipuram R, Alikhan A. Examining the race-specific prevalence of hidradenitis suppurativa at a large academic center; results from a retrospective chart review. Dermatol Online J. 2017 Jun 15;23(6).[Abstract][Full Text]
18. Saunte DM, Boer J, Stratigos A, et al. Diagnostic delay in hidradenitis suppurativa is a global problem. Br J Dermatol. 2015 Dec;173(6):1546-9.[Abstract]
19. Sabat R, Jemec GBE, Matusiak Ł, et al. Hidradenitis suppurativa. Nat Rev Dis Primers. 2020 Mar 12;6(1):18.[Abstract]
20. Phan K, Charlton O, Smith SD. Hidradenitis suppurativa and metabolic syndrome - systematic review and adjusted meta-analysis. Int J Dermatol. 2019 Oct;58(10):1112-7.[Abstract]
21. Tzellos T, Zouboulis CC, Gulliver W, et al. Cardiovascular disease risk factors in patients with hidradenitis suppurativa: a systematic review and meta-analysis of observational studies. Br J Dermatol. 2015 Nov;173(5):1142-55.[Abstract]
22. Vazquez BG, Alikhan A, Weaver AL, et al. Incidence of hidradenitis suppurativa and associated factors: a population-based study of Olmsted County, Minnesota. J Invest Dermatol. 2013 Jan;133(1):97-103.[Abstract][Full Text]
23. Wright S, Strunk A, Garg A. New-onset depression among children, adolescents, and adults with hidradenitis suppurativa. J Am Acad Dermatol. 2020 Nov;83(5):1360-6.[Abstract]
24. Jørgensen AR, Yao Y, Ghazanfar MN, et al. Burden, predictors and temporal relationships of comorbidities in patients with hidradenitis suppurativa: a hospital-based cohort study. J Eur Acad Dermatol Venereol. 2020 Mar;34(3):565-73.[Abstract]
25. Jørgensen AR, Aarestrup J, Baker JL, et al. Association of birth weight, childhood body mass index, and height with risk of hidradenitis suppurativa. JAMA Dermatol. 2020 Jul 1;156(7):746-53.[Abstract][Full Text]
26. Acharya P, Mathur M. Hidradenitis suppurativa and smoking: a systematic review and meta-analysis. J Am Acad Dermatol. 2020 Apr;82(4):1006-11.[Abstract][Full Text]
27. Garg A, Papagermanos V, Midura M, et al. Incidence of hidradenitis suppurativa among tobacco smokers: a population-based retrospective analysis in the USA. Br J Dermatol. 2018 Mar;178(3):709-14.[Abstract]
28. Kurzen H, Kurokawa I, Jemec GB, et al. What causes hidradenitis suppurativa? Exp Dermatol. 2008;17:455-456.[Abstract][Full Text]
29. Ingram JR. The genetics of hidradenitis suppurativa. Dermatol Clin. 2016 Jan;34(1):23-8.[Abstract]
30. Dufour DN, Emtestam L, Jemec GB. Hidradenitis suppurativa: a common and burdensome, yet under-recognised, inflammatory skin disease. Postgrad Med J. 2014 Apr;90(1062):216-21; quiz 220.[Abstract][Full Text]
31. Schrader AM, Deckers IE, van der Zee HH, et al. Hidradenitis suppurativa: a retrospective study of 846 Dutch patients to identify factors associated with disease severity. J Am Acad Dermatol. 2014 Sep;71(3):460-7.[Abstract]
32. Li A, Peng Y, Taiclet LM, et al. Analysis of hidradenitis suppurativa-linked mutations in four genes and the effects of PSEN1-P242LfsX11 on cytokine and chemokine expression in macrophages. Hum Mol Genet. 2019 Apr 1;28(7):1173-82.[Abstract][Full Text]
33. Wang Z, Yan Y, Wang B. γ-Secretase genetics of hidradenitis suppurativa: a systematic literature review. Dermatology. 2021;237(5):698-704.[Abstract][Full Text]
34. Hunger RE, Laffitte E, Läuchli S, et al. Swiss practice recommendations for the management of hidradenitis suppurativa/acne inversa. Dermatology. 2017;233(2-3):113-9.[Abstract][Full Text]
35. von der Werth JM, Williams HC. The natural history of hidradenitis suppurativa. J Eur Acad Dermatol Venereol. 2000;14:389-392.[Abstract]
36. Alikhan A, Sayed C, Alavi A, et al. North American clinical management guidelines for hidradenitis suppurativa: A publication from the United States and Canadian Hidradenitis Suppurativa Foundations: Part I: Diagnosis, evaluation, and the use of complementary and procedural management. J Am Acad Dermatol. 2019 Jul;81(1):76-90.[Abstract][Full Text]
37. Dauden E, Lazaro P, Aguilar MD, et al. Recommendations for the management of comorbidity in hidradenitis suppurativa. J Eur Acad Dermatol Venereol. 2018 Jan;32(1):129-44.[Abstract]
38. Nielsen VW, Jørgensen AR, Thomsen SF. Fatal outcome of malignant transformation of hidradenitis suppurativa: a case report and literature review. Clin Case Rep. 2020 Mar;8(3):504-7.[Abstract][Full Text]
39. Lee SJ, Lim JM, Lee SH, et al. Invasive cutaneous squamous cell carcinoma arising from chronic hidradenitis suppurativa: a case report of treatment by slow mohs micrographic surgery. Ann Dermatol. 2021 Feb;33(1):68-72.[Abstract][Full Text]
40. Poveda I, Vilarrasa E, Martorell A, et al. Serum zinc levels in hidradenitis suppurativa: a case-control study. Am J Clin Dermatol. 2018 Oct;19(5):771-7.[Abstract]
41. Martorell A, García FJ, Jiménez-Gallo D, et al. Update on hidradenitis suppurativa (part II): treatment. Actas Dermosifiliogr. 2015;106:716-724.[Abstract][Full Text]
42. Ingram JR, Collier F, Brown D, et al. British Association of Dermatologists guidelines for the management of hidradenitis suppurativa (acne inversa) 2018. Br J Dermatol. 2019 May;180(5):1009-17.[Abstract][Full Text]
43. Alikhan A, Sayed C, Alavi A, et al. North American clinical management guidelines for hidradenitis suppurativa: a publication from the United States and Canadian Hidradenitis Suppurativa Foundations: Part II: Topical, intralesional, and systemic medical management. J Am Acad Dermatol. 2019 Jul;81(1):91-101.[Abstract][Full Text]
44. Zouboulis CC, Bechara FG, Dickinson-Blok JL, et al. Hidradenitis suppurativa/acne inversa: a practical framework for treatment optimization - systematic review and recommendations from the HS ALLIANCE working group. J Eur Acad Dermatol Venereol. 2019 Jan;33(1):19-31.[Abstract][Full Text]
45. Chahine AA, Nahhas AF, Braunberger TL, et al. Ertapenem rescue therapy in hidradenitis suppurativa. JAAD Case Rep. 2018 Jun;4(5):482-3.[Abstract]
46. Join-Lambert O, Coignard-Biehler H, Jais JP, et al. Efficacy of ertapenem in severe hidradenitis suppurativa: a pilot study in a cohort of 30 consecutive patients. J Antimicrob Chemother. 2016 Feb;71(2):513-20.[Abstract][Full Text]
47. Riis PT, Boer J, Prens EP, et al. Intralesional triamcinolone for flares of hidradenitis suppurativa (HS): a case series. J Am Acad Dermatol. 2016 Dec;75(6):1151-5.[Abstract]
48. Leiphart P, Ma H, Naik HB, et al. The effect of antimicrobial washes on antibacterial resistance in hidradenitis suppurativa lesions. J Am Acad Dermatol. 2019 Mar;80(3):821-2.[Abstract][Full Text]
49. Jemec GB, Wendelboe P. Topical clindamycin versus systemic tetracycline in the treatment of hidradenitis suppurativa. J Am Acad Dermatol. 1998;39:971-4.[Abstract]
50. Kromann CB, Ibler KS, Kristiansen VB, et al. The influence of body weight on the prevalence and severity of hidradenitis suppurativa. Acta Derm Venereol. 2014 Sep;94(5):553-7.[Abstract][Full Text]
51. Gener G, Canoui-Poitrine F, Revuz JE, et al. Combination therapy with clindamycin and rifampicin for hidradenitis suppurativa: a series of 116 consecutive patients. Dermatology. 2009;219:148-54.[Abstract]
52. Schneller-Pavelescu L, Vergara-de Caso E, Martorell A, et al. Interruption of oral clindamycin plus rifampicin therapy in patients with hidradenitis suppurativa: An observational study to assess prevalence and causes. J Am Acad Dermatol. 2019 May;80(5):1455-7.[Abstract][Full Text]
53. Zouboulis CC, Desai N, Emtestam L, et al. European S1 guideline for the treatment of hidradenitis suppurativa/acne inversa. J Eur Acad Dermatol Venereol. 2015 Apr;29(4):619-44.[Abstract]
54. Mendes-Bastos P, Macedo R, Duarte R. Treatment of hidradenitis suppurativa with rifampicin: have we forgotten tuberculosis? Br J Dermatol. 2017 Oct;177(4):e150-e151.[Abstract]
55. Yazdanyar S, Boer J, Ingvarsson G, et al. Dapsone therapy for hidradenitis suppurativa: a series of 24 patients. Dermatology. 2011;222(4):342-6.[Abstract]
56. Nikolakis G, Kyrgidis A, Zouboulis CC. Is there a role for antiandrogen therapy for hidradenitis suppurativa? A systematic review of published data. Am J Clin Dermatol. 2019 Aug;20(4):503-13.[Abstract]
57. Boer J, van Gemert MJ. Long-term results of isotretinoin in the treatment of 68 patients with hidradenitis suppurativa. J Am Acad Dermatol. 1999;40:73-6.[Abstract]
58. Boer J, Nazary M. Long-term results of acitretin therapy for hidradenitis suppurativa. Br J Dermatol. 2010;164:170-5.[Abstract]
59. Blok JL, van Hattem S, Jonkman MF, et al. Systemic therapy with immunosuppressive agents and retinoids in hidradenitis suppurativa: a systematic review. Br J Dermatol. 2013;168:243-52.[Abstract]
60. van der Zee HH, Prens EP, Boer J. Deroofing: a tissue-saving surgical technique for the treatment of mild to moderate hidradenitis suppurativa lesions. J Am Acad Dermatol. 2010;63:475-480.[Abstract]
61. Tierney E, Mahmoud BH, Hexsel C, et al. Randomized control trial for the treatment of hidradenitis suppurativa with a neodymium-doped yttrium aluminium garnet laser. Dermatol Surg. 2009;35:1188-1198.[Abstract]
62. Mahmoud BH, Tierney E, Hexsel CL, et al. Prospective controlled clinical and histopathologic study of hidradenitis suppurativa treated with the long-pulsed neodymium:yttrium-aluminium-garnet laser. J Am Acad Dermatol. 2010;62:637-645.[Abstract]
63. Hazen PG, Hazen BP. Hidradenitis suppurativa: successful treatment using carbon dioxide laser excision and marsupialization. Dermatol Surg. 2010;36:208-213.[Abstract]
64. Kimball AB, Okun MM, Williams DA, et al. Two phase 3 trials of adalimumab for hidradenitis suppurativa. N Engl J Med. 2016;375:422-34.[Abstract][Full Text]
65. Tzanetakou V, Kanni T, Giatrakou S, et al. Safety and efficacy of anakinra in severe hidradenitis suppurativa: a randomized clinical trial. JAMA Dermatol. 2016 Jan;152(1):52-9. [Abstract][Full Text]
66. Molinelli E, Brisigotti V, Simonetti O, et al. Efficacy and safety of topical resorcinol 15% as long-term treatment of mild-to-moderate hidradenitis suppurativa: a valid alternative to clindamycin in the panorama of antibiotic resistance. Br J Dermatol. 2020 Dec;183(6):1117-9.[Abstract]
67. Pascual JC, Encabo B, Ruiz de Apodaca RF, et al. Topical 15% resorcinol for hidradenitis suppurativa: An uncontrolled prospective trial with clinical and ultrasonographic follow-up. J Am Acad Dermatol. 2017 Dec;77(6):1175-8.[Abstract]
68. Blok JL, Li K, Brodmerkel C, et al. Ustekinumab in hidradenitis suppurativa: clinical results and a search for potential biomarkers in serum. Br J Dermatol. 2016 Apr;174(4):839-46.[Abstract]
69. Takeda K, Kikuchi K, Kanazawa Y, et al. Ustekinumab treatment for hidradenitis suppurativa. J Dermatol. 2019 Dec;46(12):1215-8.[Abstract]
70. Kashetsky N, Mufti A, Alabdulrazzaq S, et al. Treatment outcomes of IL-17 inhibitors in hidradenitis suppurativa: a systematic review. J Cutan Med Surg. 2021 Aug [Epub ahead of print].[Abstract]
71. ClinicalTrials.gov. Secukinumab/hidradenitis suppurativa phase 3 interventional studies [internet publication].[Full Text]
72. Babbush KM, Andriano TM, Cohen SR. Antiandrogen therapy in hidradenitis suppurativa: finasteride for females. Clin Exp Dermatol. 2021 Jul 14 [Epub ahead of print].[Abstract]
73. Ingram JR, Woo PN, Chua SL, et al. Interventions for hidradenitis suppurativa. Cochrane Database Syst Rev. 2015;(10):CD010081.[Abstract][Full Text]
74. John H, Manoloudakis N, Stephen Sinclair J. A systematic review of the use of lasers for the treatment of hidradenitis suppurativa. J Plast Reconstr Aesthet Surg. 2016 Oct;69(10):1374-81.[Abstract]
75. Montero-Vilchez T, Diaz-Calvillo P, Rodriguez-Pozo JA, et al. The burden of hidradenitis suppurativa signs and symptoms in quality of life: systematic review and meta-analysis. Int J Environ Res Public Health. 2021 Jun 22;18(13):6709.[Abstract][Full Text]
76. Patel ZS, Hoffman LK, Buse DC, et al. Pain, psychological comorbidities, disability, and impaired quality of life in hidradenitis suppurativa [corrected]. Curr Pain Headache Rep. 2017 Nov 1;21(12):49.[Abstract]
77. Moschella SL. Hidradenitis suppurativa: complications resulting in death. JAMA. 1966;198:201-203.[Abstract]
78. Faye O, Petit F, Poli F, et al. Lymphedema as a complication of hidradenitis suppurativa in three patients [in French]. Ann Dermatol Venereol. 2007;134:567-569.[Abstract]
79. Maclean GM, Coleman DJ. Three fatal cases of squamous cell carcinoma arising in chronic perineal hidradenitis suppurativa. Ann R Coll Surg Engl. 2007;89:709-712.[Abstract][Full Text]

Guidelines

Diagnostic

North American clinical management guidelines for hidradenitis suppurativa part I: Diagnosis, evaluation, and the use of complementary and procedural management [36]
Summary
Addresses the role of diagnostic testing in evaluating patients with HS.
Published by
US Hidradenitis Suppurativa Foundation; Canadian Hidradenitis Suppurativa Foundation
Published
2019

Treatment

North American clinical management guidelines for hidradenitis suppurativa part II: Topical, intralesional, and systemic medical management [43]
Summary
Management consists of both medical and surgical approaches, often combined for best outcomes.
Published by
US Hidradenitis Suppurativa Foundation; Canadian Hidradenitis Suppurativa Foundation
Published
2019
Hidradenitis suppurativa/acne inversa: a practical framework for treatment optimization - systematic review and recommendations from the HS ALLIANCE working group [44]
Summary
Consensus recommendations for the management of patients with HS.
Published by
HS ALLIANCE working group
Published
2019
Guidelines for the management of hidradenitis suppurativa (acne inversa)[42]
Summary
Evidence-based recommendations for the management of hidradenitis suppurativa.
Published by
British Association of Dermatologists
Published
2019
European S1 guideline for the treatment of hidradenitis suppurativa/acne inversa (acne inversa)[53]
Summary
Disease should be treated based on its individual subjective impact and objective severity.
Published by
European Academy of Dermatology and Venereology
Published
2015

Credits

Authors

Topic last updated: 2022-01-07

David Cassarino, MD, PhD

Consultant Dermatopathologist

Southern California Permanente Medical Group

Los Angeles Medical Center

Los Angeles

[disclosures]

Acknowledgements:

Dr David Cassarino would like to gratefully acknowledge Dr Robert A. Lee, the previous contributor to this topic.

RAL declares that he has no competing interests.

[disclosures]

Peer Reviewers

David R. Adams, MD, PharmD

Associate Professor of Dermatology

Penn State Hershey Medical Center

Hershey

[disclosures]

Ravi Ubriani, MD, FAAD

Assistant Professor of Clinical Dermatology

Columbia University

New York

[disclosures]

Andrew Carmichael, MB, BS

Consultant Dermatologist

James Cook University Hospital

Middlesbrough

[disclosures]

Patient Instructions

Physical occlusion is thought to play a role in worsening of the disease. Thus, loose-fitting clothing and cool environments are desirable.

Counsel smoking cessation and weight loss.[36] Weight loss is important to reduce the amount of redundant skin at intertriginous areas. Smoking cessation may help reduce disease activity.

Patients should be given the details of patient support groups at diagnosis including Hope for HS.

Diseases

Highlights & Basics

Quick Reference

Definition

Classifications

Vignette

Epidemiology

Etiology

Pathophysiology

Images

Diagnostic Approach

History

Physical exam

Investigations

Risk Factors

obesity

female sex

smoking

positive family history

African-American

History & Exam

at-risk demographic

history of acne vulgaris

recurrent disease

poor response to previous antibiotic therapy

open comedones in intertriginous (axilla, groin, perineum, or inframammary) areas

nodules or abscess

symmetrical distribution

sinus tracts with scarring

premenstrual flare

Tests

bacterial culture

skin biopsy

Differential Diagnosis

Acne vulgaris

Crohn disease

Inverse psoriasis

Epidermal inclusion cyst

Furunculosis

Lymphogranuloma venereum

Squamous cell carcinoma

Criteria

Treatment Approach

Acute symptoms

Ongoing management: mild disease (Hurley Stage I)

Ongoing management: moderate disease (Hurley Stage II)

Ongoing management: severe disease (Hurley Stage III)

Treatment Options

acute abscess

mild (Hurley stage I)

moderate (Hurley stage II)

severe (Hurley stage III)

Emerging Tx

Anakinra

Topical resorcinol

Ustekinumab

Secukinumab

Finasteride

Laser or light-based therapy

Follow-Up Overview

Prognosis

Monitoring

Complications

scarring

localized infection/cellulitis

sepsis

squamous cell carcinoma

lymphedema

Citations

Key Articles

Other Online Resources

Referenced Articles

Guidelines

Diagnostic

Summary

Treatment

Summary

Summary

Summary