Navigating Hidradenitis Suppurativa Chronicles

A recent Dermatology Times® Partner Perspectives video series titled “Navigating Hidradenitis Suppurativa Chronicles” was led by Tiffany Mayo, MD, Associate Professor, Vice Chair of Diversity and Inclusion, and Director of the Clinical Research Unit at the Heersink School of Medicine department of dermatology at the University of Alabama at Birmingham. In the video series, Dr Mayo reviewed the etiology of hidradenitis suppurativa (HS), challenges associated with diagnosis, assessment of disease severity and progressive stages, and the role of biologics in treatment.

HS is a chronic, disabling skin disorder that affects approximately 0.053% to 4.10% of the general population.^1,2 It typically involves the terminal follicular unit of apocrine gland-bearing skin located in the axilla and groin region, and it is characterized by nodules, abscesses, and purulent drainage.^1,3 ““The pathophysiology of HS is not fully known,” shared Dr Mayo; however, she noted that current understanding of its mechanisms can help elucidate the way by which the disease progresses.⁴ Genetic and environmental factors may contribute to perifollicular inflammation, which leads to follicular occlusion, eventual rupture, and further inflammation.^3-5 Cytokines such as interleukin (IL)-17, IL-23, IL-1β, IL-1α, IL-12, and tumor necrosis factor (TNF)-α contribute to the inflammation and tissue damage associated with HS onset and progression.⁶

“The burden of HS is quite high,” ⁷ shared Dr Mayo, with 62% of patients experiencing painful abscesses that do not subside.⁸ Chronic pain is the most bothersome self-reported symptom associated with HS, which can contribute to sleep disturbance.^9,10 Patients with HS also frequently experience depression, anxiety, and other mental health issues.^10,11 Overall, this population is at a higher risk for suicide, and 40% of patients with HS are unable to work.^10,11 HS has a greater impact on quality of life than do other high-burden skin diseases such as alopecia, acne, psoriasis, urticaria, and atopic dermatitis.⁷

Patients receive an HS diagnosis via clinical evaluation rather than laboratory confirmation, shared Dr Mayo.¹² An accurate diagnosis is often delayed due to various diagnostic challenges, such as the presence of lesions in subcutaneous skin layers; the average time from symptom onset to accurate diagnosis is 7 to 10 years.^13-15 “At this point the disease has typically reached a severe stage,” said Dr Mayo.¹ “A delayed diagnosis, thereby a delay in appropriate treatment, can result in uncontrolled disease activity, including fibrosis and scarring, and sinus tract formation. At this stage, the scarring and tunnels are often irreversible.”¹ In addition, patients may develop resistance to treatment.¹⁵

Dr Mayo underscored the importance of early diagnosis and treatment. “Early treatment targets reversible lesions, and it may be associated with improved treatment outcomes,” she said. “Importantly, treating during [this] ‘window of opportunity’ may halt the progression of HS before patients develop irreversible scars and/or diminished quality of life.”¹⁶

The stages through which HS progresses are categorized as mild, moderate, and severe, according to Dr Mayo.^1,4 These stages are defined by the extent to which lesions cover each area and the degree and duration of pain.^1,17 The mild stage can present with isolated, deep, painful nodules that may evolve to single or multiple abscesses without sinus tracts or scarring.¹⁶ During this stage, “periods of remission may last several months,” said Dr Mayo, “and the clinical aspects of the skin may be normal.” She noted that misdiagnosis occurs frequently at this stage.^1,17 In the moderate stage, HS has progressed to recurrent abscesses and widely separated lesions that may be restricted to a certain body region or multiple areas. Sinus tract formation and scarring may be present.^1,17,18 Finally, at the severe stage, HS presents with diffuse lesions in the affected regions as well as interconnected tracts, abscesses, and ruptured nodules. “Patients experience ongoing pain and discomfort at this stage,” said Dr Mayo.^1,18 Again, she highlighted the importance of early treatment. “If [left] untreated, HS can progress [to] interconnected abscesses that can ultimately result in keloids, contractures, and immobility.” ^1,17

Dr Mayo emphasized that the clinical presentation of HS may not always accurately represent disease severity, and she suggested that subclinical disease may be more accurately identified with ultrasound or more powerful tools such as magnetic resonance imaging (MRI).¹² In addition, patient assessments such as the Hidradenitis Suppurativa Impact Assessment and Hidradenitis Suppurativa Symptom Assessment could provide valuable insight for staging HS.¹⁸

The goal of HS treatment is to address current symptoms and to prevent progression.¹ In addition, psychiatric and medical comorbidities should be managed.¹⁹ Treatment is selected based on HS stage as well as factors unique to individual patients and their preferences.^1,10,12,20 “Patients may feel as if they have already tried the recommended treatments or have hesitancy toward some treatments,” advised Dr Mayo. “[However,] clinicians should still try to educate patients on the overall treatment plan and review the risk[s] and benefits of treatment. . . .This allows patients to make a well-informed decision regarding their health.”^14,15 Patient visits can be time-intensive, involving extensive counseling and additional time for prior authorization of medications.²¹ Resources to support clinicians are available through the HS foundations, such as prior authorization templates, treatment guidelines, and HS specialty clinic directories.²²

Clinical management is challenging and consists of both medical (eg, topicals, systemic antibiotics, systemic immunomodulators, retinoids, hormonal therapies, and biologics) and procedural (eg, laser, excisions and deroofing) approaches, which must often be combined for best outcomes.^1,15,19 Dr Mayo focused her discussion on systemics, noting that systemics were typically used to treat moderate to severe HS, which are the stages at which HS is usually diagnosed.^1,15 “Standard systemic agents have been associated with varying levels of success in treating moderate to severe HS,” ¹⁹ she said. “This highlights the need for therapies with improved efficacy and safety profiles.” For example, antibiotics have historically been used as a mainstay treatment; however, Dr Mayo advised that they should be used only as adjunctive therapy in severe cases, with consideration to their low response rates, recurrence following cessation, and risk of antibiotic resistance.¹⁹

Similarly, she said that hormone therapies, which are also used frequently, can be given as adjunctive treatment in patients with severe HS. Dr Mayo noted that limited evidence supports hormone therapy efficacy, and that progesterone can exacerbate HS among some patients.¹⁹ Retinoids, and certain immunomodulatory therapies (eg, methotrexate, azathioprine, and cyclosporine), may also be used to treat HS; however, they have demonstrated low efficacy and concerning side effects.¹⁹ Pulsed or tapered prednisone has been used as either an acute rescue therapy or a bridge to other therapy with considerable success but must be limited due to side effects.¹⁹ Dr Mayo shared the value of combination therapy.^2,19 “Personally, most of my patients are on combination therapy tailored to their symptoms,” she said. Currently, data regarding combination therapy are limited.¹⁵

Biologics have recently shown promise as a treatment option for HS.¹⁹ “[Biologics have] demonstrated preliminary success in the management of other inflammatory diseases such as rheumatoid arthritis and psoriasis,” said Dr Mayo.²³ “The North American clinical management guidelines for HS have recognized the potential therapeutic value offered by biologics, stating that biologics are becoming the cornerstone therapy for moderate to severe HS.”¹⁹ At this time, primary limitations in research of biologics include considerable variability of end points, lack of dose-ranging studies, and short follow-up periods.¹⁹

In studies, biologic targets of interest have included TNF, IL-1, IL-12, IL-17, and IL-23 pathways. Biologics currently approved for moderate to severe HS include a TNF-α inhibitor and an IL-17A inhibitor.¹⁹ “Biologic therapies can target preclinical and subclinical lesions in addition to clinically visible lesions, [which] is an important consideration,” said Dr Mayo.¹⁰ “Remember, a goal in managing HS is to prevent disease progression, sinus tract formation, and fibrosis.”¹ She reiterated the negative effects HS can have on patient quality of life, including its higher risk of depression and suicide than that of the general population.¹⁰ “As HCPs, we should take this risk seriously,” she said. Delay in initiation of biologic therapy may be a missed opportunity to achieve disease control in patients with moderate-to-severe HS.²⁴

In summary, Dr Mayo described how HS is a chronic, painful diseasewith a substantial impact on patient quality of life.^1,7 There is typically a delay in time to accurate diagnosis, and management is often suboptimal.^13-15,19 “Though more work is needed, we can support our patients by helping them understand the disease,” she said. “It is important to name the condition and help the patient understand how and why it needs to be treated.” In addition to patient education, collaboration with local HS experts or other physicians can play an important role in disease management.²² “We are all responsible for ensuring that patients are accurately diagnosed and are on the right track,” said Dr Mayo.

References

1. Kimball AB, Jemec GBE, eds. Hidradenitis Suppurativa: A Disease Primer. Adis; 2017.
2. Gill L et al. F1000Prime Rep. 2014;6:112.
3. Saunte DML, Jemec GBE. JAMA. 2017;318(20):2019-2032.
4. Jemec GBE. N Engl J Med. 2012;366(2):158-164.
5. Goldburg SR et al. J Am Acad Dermatol. 2020;82(5):1045-1058.
6. Hunt A et al. Dermatol Ther (Heidelb). 2023;13(7):1391-1407.
7. Dufour DN et al. Postgrad Med J. 2014;90(1062):216-221.
8. von der Werth JM, Williams HC. J Eur Acad Dermatol Venereol. 2000;14(5):389-392.
9. Vossen ARJV et al. Front Immunol. 2018;9:2965.
10. Sabat R et al. Nat Rev Dis Primers. 2020;6(1):18.
11. Kimball AB et al. J Eur Acad Dermatol Venereol. 2020;34(6):1302-1308.
12. Mendes-Bastos P et al. Br J Dermatol. 2023;188(5):591-600.
13. Atzori L et al. Clinical Dermatology. 2017;5(1):1-4.
14. Garg A et al. J Am Acad Dermatol. 2020;82(2):366-376.
15. Snyder CL et al. Clin Cosmet Investig Dermatol. 2023;16:1833-1841.
16. Marzano AV et al. Br J Dermatol. 2021;184(1):133-140.
17. Poli F et al. Clinical presentation. In: Jemec GBE et al, eds. Hidradenitis Suppurativa. Springer; 2006:11-24.
18. Alikhan A et al. J Am Acad Dermatol. 2019;81(1):76-90.
19. Alikhan A et al. J Am Acad Dermatol. 2019;81(1):91-101.
20. Willems D et al. Patient. 2023;16(2):153-164.
21. Flood KS et al. J Am Acad Dermatol. 2021;84(3):e155-e160.
22. HS Foundation. Accessed May 6, 2024. https://www.hs-foundation.org/
23. Lee RA, Eisen DB. J Am Acad Dermatol. 2015;73(5)(suppl 1):S82-S88.
24. Ring HC et al. Br J Dermatol. 2022;187(4):523-530.