Abstract
Hidradenitis suppurativa is a chronic, progressive inflammatory disease of the skin with many systemic implications. Hidradenitis suppurativa is frequently underdiagnosed or misdiagnosed, particularly because of heterogeneity in presentation and low disease recognition. Patients can see multiple types of health care providers, including primary care providers, along their journey to an accurate diagnosis. This review provides a comprehensive overview of the clinical presentation, associated comorbidities, and life impact associated with hidradenitis suppurativa. Disease features described here can facilitate earlier identification of hidradenitis suppurativa, differentiation from common mimickers, and timely referrals for multidisciplinary management when needed. Engagement of the medical community will also support comprehensive care strategies necessary in hidradenitis suppurativa.
Keywords
Clinical Significance
- •Hidradenitis suppurativa is a progressive inflammatory skin disease that is often underdiagnosed or misdiagnosed because of poor recognition in the medical community.
- •Increased awareness of the clinical presentation, associated comorbidities, and quality of life impact can help address unmet needs for patients.
- •This practical guide provides detailed information that can support timely hidradenitis suppurativa diagnosis and care strategies by primary care providers.
Statement of Literature Search
This review is based on previously published studies and does not contain novel data. This narrative review included publications identified from a series of PubMed searches from June 2021 through January 2022. A general internet search for relevant resources on hidradenitis suppurativa was also conducted to include useful resources for providers and patients to access. References detailing information on the hidradenitis suppurativa population, clinical presentation, diagnosis, common comorbidities and screenings, impact on patient quality of life and mental health, understanding of hidradenitis suppurativa pathogenesis, therapeutic options, and management were included. Based on the authors’ judgement, publications determined to be irrelevant were excluded from consideration. Any relevant references cited within the publications discussed in this review and articles known by the authors were also considered.
Introduction to Hidradenitis Suppurativa
Hidradenitis suppurativa is a chronic, progressive, and potentially debilitating inflammatory skin disease characterized by painful nodules, abscesses, draining tunnels, and disfiguring scars.
1.
Prevalence ranges from 0.1% to 2.0% in the United States and Europe,- 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; 81: 76-90
2
, 3
, 4
and the incidence in the United States is approximately 11.4 cases per 100,000 population.5
,6
Standardized prevalence is highest among patients aged 18 to 40 years.4
,6
,7
Hidradenitis suppurativa onset most commonly occurs in early adulthood, and onset prior to adrenarche is rare.8
Additionally, hidradenitis suppurativa has a bimodal distribution and is also known to occur in midlife.6
Hidradenitis suppurativa predominantly affects women,4
,9
and Black Americans are disproportionately affected compared with the general population.10
,11
Pain, drainage, and odor are among the most burdensome symptoms for patients with hidradenitis suppurativa. In a prospective multinational survey, approximately 60% of patients reported recent hidradenitis suppurativa-related pain as “moderate or higher,” while nearly 5% described recent pain to be “the worst possible.”12
Additionally, malodorous drainage frequently requires management. The negative impact of hidradenitis suppurativa on quality of life due to the physical, psychosocial, and comorbid burden of the disease is substantial.13
,14
In part, because of poor recognition of hidradenitis suppurativa within the medical community, the average time from onset of symptoms to diagnosis is approximately 10 years.
12
During this period, patients may experience disease progression that can lead to disfigurement and disability. Additionally, patients often experience fragmented care for flare management at acute and ambulatory care facilities with a variety of providers.15
, 16
, 17
There is an opportunity for primary care and specialty providers to support timely recognition of hidradenitis suppurativa and screening and management for the numerous comorbid conditions associated with the disease.18
,19
We provide a comprehensive review of the clinical manifestations, associated comorbidities, and life impact of hidradenitis suppurativa to facilitate early recognition and diagnosis of hidradenitis suppurativa and insights into pathogenesis and therapeutic options.Recognizing the Clinical Presentation of Hidradenitis Suppurativa
In the absence of a diagnostic biomarker, hidradenitis suppurativa remains a clinical diagnosis defined by 3 major diagnostic criteria: 1) presence of inflammatory nodules, abscesses, and tunnels (fistula or sinus) on the skin; 2) involvement of typical anatomic sites—axillae, inframammary and intermammary folds, inguinal creases, perineal region, and buttocks; and 3) recurrence and chronicity (Figure, A).
1.
,- 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; 81: 76-90
20
,21
Inflamed nodules and abscesses are often erythematous and tender, with abscesses showing warmth and fluctuance.21
Tunnels may open to the skin surface and form coalescing and interconnecting tracts within the dermis.22
Drainage of malodorous material containing cellular debris, microbes, and pus may be seen.23
Culture of lesions or drainage commonly reveals polymicrobial content or normal skin flora. Hidradenitis suppurativa is an inflammatory disease, and the potential role of the skin microbiome on the surface or in the dermis of the skin is not yet well established.24
Some antibiotic strategies have utility in the management of hidradenitis suppurativa; however, it is not yet clear whether their effectiveness relates to their anti-inflammatory or antimicrobial properties.25
Typical hidradenitis suppurativa lesions may be solitary or multiple, and the chronicity of the disease results in significant scarring and disfigurement (Figure, A).22
,26
Hidradenitis suppurativa is frequently misdiagnosed as cutaneous abscess, inflamed epidermal cyst, furunculosis, cellulitis, and occasionally, necrotizing fasciitis;26
,27
however, recurrent episodes of lesions favoring folds of the body help to distinguish hidradenitis suppurativa from these other entities.
Figure(A) Clinical features of hidradenitis suppurativa lesions and (B) a schematic of the pathogenesis of hidradenitis suppurativa.
Show full caption
IFN = interferon; IL = interleukin; TGFβ = transforming growth factor β; Th = T helper; TNF = tumor necrosis factor.
Common Comorbidities in Hidradenitis Suppurativa
The chronic inflammatory state observed in hidradenitis suppurativa is associated with upregulation of tissue and circulating cytokines, including tumor necrosis factor (TNF) and interleukin (IL)-1, IL-6, and IL-17, among others. Inflammatory cytokine upregulation has been hypothesized as a pathogenic link to several associated comorbid conditions.
18
A cross-sectional analysis of electronic health record data for patients with hidradenitis suppurativa and matched controls found that patients with hidradenitis suppurativa had significantly higher overall comorbidity burden.19
Hidradenitis suppurativa has also been associated with an adjusted increase in all-cause mortality risk of 14% compared with healthy controls.28
In addition to recognizing the disease early in its course, primary and specialty physicians have a critical role in the interdisciplinary management of patients with hidradenitis suppurativa who require comprehensive comorbidity screening and mitigation strategies.Comorbid conditions associated with hidradenitis suppurativa include endocrine
29
,30
and metabolic-related conditions,30
, 31
, 32
, 33
, 34
, 35
, 36
, 37
cardiovascular disease,38
,39
obstetric risks,40
gastrointestinal disorders,41
,42
musculoskeletal diseases,43
,44
and—perhaps most importantly—mental health disorders18
,45
, 46
, 47
, 48
, 49
, 50
as well as other comorbidities (Table 1).37
,51
, 52
, 53
, 54
, 55
A comprehensive comorbidity screening strategy for patients with hidradenitis suppurativa is critical to achieving and maintaining overall health. Primary care providers have an important role in managing and coordinating care for the treatment of these conditions to help manage and improve hidradenitis suppurativa disease burden.Table 1Common Comorbidities Associated with Hidradenitis Suppurativa
| Comorbid Condition | Increased Prevalence or Odds Among Patients with Hidradenitis Suppurativa |
|---|---|
| Polycystic ovary syndrome |
|
| Metabolic syndrome and related conditions |
|
| Dyslipidemia |
|
| Diabetes mellitus | |
| Obesity |
|
| Cardiovascular events |
|
| Obstetrics risks |
|
| Inflammatory bowel disease, including ulcerative colitis and Crohn disease |
|
| Arthropathies | |
| Psychological distress and related conditions |
|
| Sexual dysfunction |
|
| Modifiable risk factors | |
| Substance use disorders |
|
| Tobacco smoking |
|
BMI = body mass index; CI = confidence interval; EHR = electronic health record; HR = hazard ratio; OR = odds ratio.
The Psychological Burden Experienced with Hidradenitis Suppurativa
Hidradenitis suppurativa symptoms—including pain, itch, drainage, and odor—often interfere with daily functions.
56
, 57
, 58
, 59
Patients also experience significant social stigma60
,61
and negative body image62
due to affected body areas, malodor, disfigurement, and associated obesity and tobacco smoking. The substantial emotional and psychological impact of hidradenitis suppurativa13
has also been associated with low self-esteem and poor body image, which can negatively impact social and intimate relationships.63
,64
The adjusted odds of depression and suicide among patients with hidradenitis suppurativa compared with those without hidradenitis suppurativa are 1.3 to 4.8 times47
,48
,65
,66
and 2.4 to 2.8 times49
,67
higher, respectively. Additionally, anxiety is more common in those with hidradenitis suppurativa.45
,67
Furthermore, the ongoing cost and challenges to access of care and increased health care resource utilization related to managing mental health disorders impose a significant financial burden on patients.68
,69
Taken together, a thorough understanding of the key clinical features of hidradenitis suppurativa, the psychological impact, and frequent comorbidities will facilitate an identification of hidradenitis suppurativa.Current Understanding of the Pathogenesis of Hidradenitis Suppurativa
The pathogenic mechanisms of hidradenitis suppurativa are not well understood; however, genetics, microbes, immune dysregulation, and hormonal fluctuations are thought to play important roles in its pathophysiology (Figure, B).
Retrospective cohort studies have reported that up to 40% of patients have a family history of hidradenitis suppurativa, which has also been associated with earlier disease onset.
54
,70
Mutations in the γ-secretase complex have been identified in patients with hidradenitis suppurativa and are associated with a severe, widespread clinical phenotype.71
Preclinical studies have suggested that loss-of-function mutations in γ-secretase genes may lead to initiation of follicular hyperkeratosis and cyst formation similar to hidradenitis suppurativa.71
Mutations in genes important in innate immune function have been implicated in hidradenitis suppurativa. Patients with pyogenic arthritis, pyoderma gangrenosum, and acne syndrome can have a hidradenitis suppurativa-like phenotype, and these patients have autosomal dominant mutations in PSTPIP1. Mutations in MEFV, the gene that encodes pyrin and is implicated in familial Mediterranean fever, has also been implicated in hidradenitis suppurativa. These data suggest a potential role for innate immune dysfunction in hidradenitis suppurativa pathogenesis.72
Follicular occlusion due to hyperproliferation of keratinocytes is believed to be the initiating event in hidradenitis suppurativa.
73
,74
Subsequent follicle rupture leads to the seeding of microbes and keratinocyte debris from the hair follicle into the surrounding dermis. A subsequent inflammatory response is triggered and results in the recruitment of inflammatory cells, including neutrophils and lymphocytes, leading to the development of inflammatory lesions.75
This response sustains the chronic inflammation seen with hidradenitis suppurativa.24
,76
,77
Elevated levels of circulating complement factors are proposed drivers of neutrophil recruitment and activation that cause subsequent enzyme release in hidradenitis suppurativa lesions.78
Aberrant inflammatory signaling is also thought to increase levels of pro-inflammatory cytokines, such as TNF-α, IL-1ß, IL-23, IL-12, and IL-17,79
,80
which have been observed in lesional skin and serum in patients with hidradenitis suppurativa.81
, 82
, 83
, 84
The post-adrenarche and premenopausal onset, association with polycystic ovary syndrome, and perimenstrual and peripartum disease flares suggest that hormone dysregulation may be an underlying factor in the pathogenesis of hidradenitis suppurativa.
25
,29
,85
While the direct role of sex hormones is not well understood in hidradenitis suppurativa, there is evidence to suggest that patients may experience benefit from anti-androgen therapies.25
Deepening understanding of hidradenitis suppurativa biology will facilitate identification of disease targets that may ultimately lead to development of effective novel therapeutics.Therapeutic Options and Disease Management
A multifaceted approach to care should include medical and surgical management as well as screening for and managing associated comorbidities. Given the heterogeneity in response to treatments and potential for disease progression, early referral to dermatology should be considered for patients with suspected hidradenitis suppurativa.
Medical treatments for hidradenitis suppurativa are described in Table 2. First-line treatments in early-stage hidradenitis suppurativa include topical and systemic antibiotics.
25
Topical management of early hidradenitis suppurativa includes antiseptic cleansers, including benzoyl peroxide wash, and antibiotics, although evidence supporting their efficacy is limited.21
,25
,86
Oral tetracyclines, most commonly doxycycline, as first-line systemic antibiotics for mild disease may improve symptoms via anti-inflammatory activity. Combination oral antibiotic regimens have also shown efficacy.87
,88
Therapeutic strategies for antibiotic regimens should consider the potential benefit with the risk of resistance to optimize treatment duration and frequency. While the role of androgens in hidradenitis suppurativa is unclear, anti-androgen treatments have been observed to improve symptoms.89
, 90
, 91
In women with hidradenitis suppurativa, spironolactone 100-200 mg daily is generally well tolerated and may be as effective as antibiotic strategies.Table 2Treatment Options Available for Hidradenitis Suppurativa
| Available First-Line Treatments That Can Be Administered Prior to Dermatologist Visits | |
|---|---|
| Topical therapies | Clindamycin 1% solution/gel QD-BID Washes: zinc pyrithione, chlorhexidine, benzoyl peroxide Triamcinolone (intralesional) 40 mg/mL in up to 3 lesions |
| Systemic antibiotics | Doxycycline 100 mg BID Rifampin 300 mg BID and clindamycin 300 mg BID Rifampin 300 mg BID plus moxifloxacin 400 mg QD and metronidazole 500 mg TID |
| Hormonal therapies | Combination contraceptives Spironolactone 100-150 mg QD (or divided BID) Metformin 500 mg QD to BID Finasteride 1.25-5 mg QD |
| Treatment considerations after diagnostic confirmation by a dermatologist | |
| Retinoids | Isotretinoin Acitretin Alitretinoin |
| Systemic immunomodulatory therapies | Cyclosporine Systemic steroids |
| Biologics | TNF-α inhibitors: adalimumab, golimumab, infliximab IL-17 inhibitors: secukinumab IL-1 inhibitors: anakinra, canakinumab IL-12/23 inhibitors: ustekinumab |
| Surgical procedures | Incision and drainage Cryosurgery Unroofing/deroofing Lesional or wide excision |
| Additional opportunities for PCPs for comprehensive disease management | |
| Patient education | Inform patients of the following:
|
BID = twice daily; IL = interleukin; PCP = primary care provider; QD = daily; TID = 3 times a day; TNF = tumor necrosis factor.
A paradigm shift in treatment strategy may be needed to focus on early referral to dermatologists (Table 3) and earlier use of biologics that appropriately address the underlying inflammation associated with hidradenitis suppurativa (Table 2). The application of biologics in hidradenitis suppurativa is an evolving area of research. However, the Food and Drug Administration-approved human monoclonal antibody against TNF-α, adalimumab, is approved for use in moderate to severe hidradenitis suppurativa.
92
Two parallel placebo-controlled phase 3 clinical trials (PIONEER 1 and 2) evaluated adalimumab in adult patients with moderate to severe hidradenitis suppurativa. The primary efficacy outcome of 50% reduction in counts of abscesses and nodules was achieved by significantly more participants who received adalimumab in PIONEER 1 (41.8% vs 26.0%) and PIONEER 2 (58.9% vs 27.6%) than by those who received placebo. Significantly greater improvements in lesion counts, pain, and modified Sartorius score for disease activity were observed in patients who received adalimumab vs placebo.93
Furthermore, in an open-label extension follow-up study, sustained improvements in hidradenitis suppurativa clinical response, lesion counts, skin pain, and Dermatology Life Quality Index score were maintained through week 168 with continuous weekly dosing with adalimumab.94
Although limited studies are available, some have demonstrated the efficacy of infliximab,95
,96
ustekinumab,97
,98
anakinra,99
,100
bimekizumab,101
and secukinumab95
for the treatment of patients with hidradenitis suppurativa. Biologic therapy centered on inhibition of inflammatory cytokines is a therapeutic option for patients with moderate and severe hidradenitis suppurativa. Improved disease management may be achieved with aggressive systemic therapies coupled with appropriate management of comorbidities.Table 3Resources for Hidradenitis Suppurativa Awareness and Education
| Hidradenitis Suppurativa Foundation | Hidradenitis Suppurativa Foundation includes information and resources for both patients and providers about education, management, and events for hidradenitis suppurativa Available online: https://www.hs-foundation.org/ Including a hidradenitis suppurativa clinic finder: https://www.hs-foundation.org/hs-specialty-clinics |
| Hidradenitis Suppurativa Source: Hidradenitis Suppurativa Clinical Resource | AbbVie has compiled an online portal that includes support and resources for both patients and providers to increase hidradenitis suppurativa awareness and education, including patient handouts and conversation guides Available online: https://www.hsdiseasesource.com/resources |
| Hope for Hidradenitis Suppurativa | Hope for Hidradenitis Suppurativa—a volunteer-based patient and provider nonprofit organization to support and advocate for patients with hidradenitis suppurativa—provides hidradenitis suppurativa resources, such as support group information, patient care information, research, and news Available online: https://hopeforhs.org/ |
| Hidradenitis Suppurativa Connect | Hidradenitis Suppurativa Connect provides resources for providers (dermatologists and general clinicians) and patients (adult and pediatric), including mental health support, community connections and events, and clinical trial recruitment information Available online: https://www.hsconnect.org/ |
| American Academy of Dermatology (AAD) | The AAD has compiled a collection of hidradenitis suppurativa information to increase education and awareness of this disease for providers and patients, including symptoms, causes, treatments, self-care, mechanisms for coping with depression, and dietary changes that may impact hidradenitis suppurativa Available online: https://www.aad.org/public/diseases/a-z/hidradenitis-suppurativa-overview |
Surgical interventions have an important role in the management of hidradenitis suppurativa. Deroofing is an ambulatory procedure that can eliminate draining tunnels that open to the surface of the skin.
102
Wide local excision is used to remove more extensive inflamed and scarred areas of skin.1.
It is important to optimize medical therapy to reduce the inflammatory burden prior to surgical interventions and to emphasize that surgical interventions are not curative or protective against hidradenitis suppurativa.- 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; 81: 76-90
25
A thorough consideration of appropriate therapeutic options and awareness of the potential to shift or combine therapeutic strategies to optimize disease management can facilitate comprehensive patient care.Comprehensive Patient Care Strategies
Screening and referral when needed for comorbid conditions, including endocrine
103
and metabolic disorders, cardiovascular disorders,104
obstetric risks,40
gastrointestinal and musculoskeletal disorders, substance misuse,105
,106
mental health disorders,107
, 108
, 109
and pain110
,111
can help patients manage their overall health (Table 4). Mental health disorders are some of the most frequent comorbidities of hidradenitis suppurativa. The Patient Health Questionnaire 2112
is a quick screening tool for depression,18
,113
which, if positive, can be followed up with the more comprehensive 9-item Patient Health Questionnaire.114
Limited data support smoking cessation and weight reduction as strategies for hidradenitis suppurativa management.115
,116
However, given the associations between hidradenitis suppurativa and smoking and obesity, it is important to counsel patients on the benefits of risk factor modifications to improve overall health outcomes.Table 4Recommended Screening Methods for Comorbidities Associated with Hidradenitis Suppurativa
| Comorbid Condition | Screening Method |
|---|---|
| Metabolic syndrome and related conditions (eg, glucose dysregulation, hypertension, dyslipidemia, and obesity) |
|
| Obesity |
|
| Diabetes mellitus |
|
| Polycystic ovary syndrome |
|
| Cardiovascular disease and related conditions |
|
| Inflammatory bowel disease |
|
| Spondyloarthritis |
|
| Depression/depressive symptoms |
|
| Generalized anxiety disorder |
|
| Suicidality/self-harm |
|
| Pain |
|
| Substance use disorder |
|
FSH = follicle-stimulating hormone; HDL = high-density lipoproteins; LH = luteinizing hormone.
Pain related to disease flares has a major impact on quality of life for patients with hidradenitis suppurativa.
110
,111
Identification and management of acute and chronic pain related to hidradenitis suppurativa can aid in the establishment of an optimal treatment plan.117
, 118
, 119
Acute pain is primarily thought to be neuropathic and typically presents as sharp, burning, stinging, or shooting pain. Acute pain can be experienced with hidradenitis suppurativa inflammatory lesions and frequently requires medical intervention.118
Chronic pain is primarily thought to be nociceptive, may be experienced with advanced hidradenitis suppurativa, and is described as gnawing, aching, or tender, and is triggered by mechanical stress.119
Topical analgesics, acetaminophen, and nonsteroidal anti-inflammatory medications can be used to treat acute pain in patients with hidradenitis suppurativa. The use of opioids may be necessary for some patients but should be used with caution due to the potential for addiction, misuse, and dependence.1.
,- 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; 81: 76-90
106
Psychological comorbidities that affect perception and tolerance of pain should be considered as part of the optimal pain management strategy for each patient. Ultimately, referral to a pain management or palliative care specialist may be necessary.Resources are available to support awareness in and education of patients with hidradenitis suppurativa related to disease course, disease impact, and opportunities to engage behaviors that may support overall health (Table 3). Patients will also benefit from understanding the inflammatory nature of the disease and the potential for immunomodulating therapies to control disease symptoms.
Conclusion
Several opportunities to improve care and outcomes for patients with hidradenitis suppurativa are available. Timely disease recognition and management are essential to controlling symptoms, mitigating disease progression, and improving quality of life for patients with hidradenitis suppurativa. Comprehensive care strategies coordinated among medical and surgical disciplines are necessary to improve the overall health of patients with hidradenitis suppurativa.
Acknowledgments
Medical writing support was provided by Charli Dominguez, PhD, CMPP, of Health Interactions, Inc, Chicago, IL, and was funded by Novartis Pharmaceuticals Corporation. This manuscript was developed in accordance with Good Publication Practice (GPP3) guidelines. Authors had full control of the content and made the final decision on all aspects of this publication.
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Article Info
Publication History
Published online: October 13, 2022
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Funding: Support for third-party writing assistance for this manuscript was provided by Novartis.
Conflicts of Interest: AG has served as an advisor and/or received honoraria from AbbVie, Aclaris Therapeutics, AnaptysBio, Aristea Therapeutics, Boehringer Ingelheim, Bristol Meyers Squibb, Incyte, InflaRx, Insmed, Janssen, Novartis, Pfizer, UCB, and Viela Biosciences; has received research grants from AbbVie, UCB, and National Psoriasis Foundation; and is co-copyright holder of the Hidradenitis Suppurativa Investigator Global Assessment and Hidradenitis Suppurativa Quality of Life instruments. HBN has received consulting fees from 23andMe, AbbVie, Aristea Therapeutics, DAVA Oncology, and Nimbus Therapeutics; advisory fees from Boehringer Ingelheim; investigator fees from Pfizer; grant support from AbbVie; serves as an associate editor for JAMA Dermatology; and is a board member of the US Hidradenitis Suppurativa Foundation. JSK has received speaker and consultant fees from AbbVie; consultant fees from ChemoCentryx, Incyte, Janssen, Novartis, and UCB; and grant support from Incyte.
Authorship: All authors made substantial contributions to the conception and methodology of this literature review; were involved in drafting and critically revising the manuscript; have given necessary attention to ensure the integrity of the work; and have read and approved all versions of this manuscript and its submission.
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