Guideline sources The following summarized guidelines for the evaluation and management of hidradenitis suppurativa are prepared by our editorial team based on guidelines from the United States Hidradenitis Suppurativa Foundation (USHSF/CHSF 2019) and the British Association of Dermatologists (BAD 2019). 1 2 3 4 4 4 5 6 7 8 Definition Hidradenitis suppurativa is a chronic inflammatory skin condition characterized by painful, suppurative lesions in apocrine gland-bearing areas. 4 Epidemiology Hidradenitis suppurativa is caused by immune dysregulation in the terminal follicular epithelium and is thought to occur due to a combination of genetic predisposition and environmental factors. 7 Pathophysiology https://web.pathway.md/diseases/recLhlEqiLh0BxVCU 1/8 6/24/23, 12:45 PM Hidradenitis suppurativa Pathway Estimates of the prevalence of hidradenitis suppurativa globally range from 0.03% to 4%. The incidence of hidradenitis suppurativa in the United States is 8.6 per 100,000 population/year. 5 6 Disease course The dysregulated innate and adaptive immune response leads to perifollicular inflammation, hyperkeratosis, and occlusion of hair follicles in apocrine gland-bearing areas. Spillage of sebum and debris occurs due to rupture of dilated hair follicles, triggering an inflammatory response and the development of painful, inflamed nodules, abscesses, fistulas, and sinus tracts. 8 Prognosis and risk of recurrence Hidradenitis suppurativa is associated with increased all-cause mortality (incidence rate ratio 1.35, 95% CI, 1.15-1.59). Recurrence rates vary according to treatment, with rates of 13%, 22%, and 27% reported following wide excision, local excision, and deroofing, respectively. 4 4 Guidelines 1. Classification and risk stratification Severity grading: As per BAD 2019 guidelines, document the Hurley stage at baseline for the worst-affected region in all patients with hidradenitis suppurativa: Situation Guidance Abscess formation, single or multiple, without sinus tracts and cicatrization Stage I Recurrent abscesses with tract formation and cicatrization, single or multiple, widely separated lesions Stage II Diffuse or near-diffuse involvement, or multiple interconnected tracts and abscesses across the entire area. E Stage III As per USHSF 2019 guidelines, use the following to assess disease severity: hurley staging inflammatory lesion counts (abscesses and inflammatory lesions) clinical performance evaluation. B Show 2 more 2. Diagnostic investigations https://web.pathway.md/diseases/recLhlEqiLh0BxVCU 2/8 6/24/23, 12:45 PM Hidradenitis suppurativa Pathway Laboratory tests: Do not obtain routine microbiologic testing unless signs of secondary infection such as surrounding cellulitis or fever are present. Recognize that a negative culture may support a diagnosis of hidradenitis suppurativa based on consensus-derived diagnostic criteria. D Do not obtain biomarker or genetic testing in patients with hidradenitis suppurativa. D Screening for comorbidities: As per BAD 2019 guidelines: Screen patients with hidradenitis suppurativa for associated comorbidities including depression, anxiety and cardiovascular risk factors (diabetes, hypertension, hyperlipidemia and central obesity). Refer patients with persistent gastrointestinal symptoms for IBD screening. E Monitor for the following conditions in patients with long-standing, moderate-to-severe hidradenitis suppurativa: fistulating gastrointestinal disease inflammatory arthritis genital lymphedema cutaneous SCC anemia. E As per USHSF 2019 guidelines, screen for smoking, diabetes, metabolic syndrome, depression/anxiety B , follicular occlusion tetrad B , and SCC of HS-affected skin B Show 2 more 3. Medical management General principles: Manage patients with hidradenitis suppurativa via a multidisciplinary team approach, particularly when considering surgical interventions. E Consider referring patients with Hurley stage III (severe) disease immediately to dermatology secondary care. E Antibiotics, systemic: As per BAD 2019 guidelines, administer oral tetracyclines such as doxycycline or lymecycline for at least 12 weeks in patients with hidradenitis suppurativa, considering treatment breaks to assess need for ongoing therapy and to limit the risk of antimicrobial resistance. A Show 3 more As per CHSF/USHSF 2019 guidelines, administer tetracyclines for 12 weeks or as long-term maintenance when appropriate in patients with mild-to-moderate hidradenitis suppurativa. B Show 6 more Antibiotics, topical: https://web.pathway.md/diseases/recLhlEqiLh0BxVCU 3/8 6/24/23, 12:45 PM Hidradenitis suppurativa Pathway As per BAD 2019 guidelines, consider offering clindamycin 1% solution in patients with hidradenitis suppurativa. C As per CHSF/USHSF 2019 guidelines, consider offering topical clindamycin to reduce pustules in patients with hidradenitis suppurativa, but recognize that it carries a high risk of bacterial resistance. C Antiandrogen therapy: As per BAD 2019 guidelines: Consider offering metformin in patients with hidradenitis suppurativa with concomitant diabetes mellitus, and in female patients with PCOS or pregnancy. E Insufficient evidence to support the use of the following agents in patients with hidradenitis suppurativa unresponsive to conventional systemic therapy: cyproterone ethinyloestradiol with cyproterone acetate ethinyloestradiol with norgestrel finasteride spironolactone. I As per USHSF 2019 guidelines: Consider administering the following hormonal agents in appropriate female patients with hidradenitis suppurativa, either as monotherapy for mild-to-moderate disease or in combination with other agents for more severe disease: estrogen-containing combined oral contraceptives (C, II) finasteride (C, III) cyproterone acetate spironolactone (C, III) metformin. (C, III) (Ungraded). Avoid progestogen-only contraceptives as they may worsen hidradenitis suppurativa. Retinoids: As per BAD 2019 guidelines, consider administering acitretin 0.3-0.5 mg/kg/day in male and non-fertile female patients with hidradenitis suppurativa unresponsive to antibiotics. C Show 2 more As per USHSF 2019 guidelines, consider administering isotretinoin only as second- or third-line therapy, or in patients with severe concomitant acne. C Show 2 more Corticosteroids: As per BAD 2019 guidelines, insufficient evidence to support the use of hydrocortisone or oral prednisolone in patients with hidradenitis suppurativa unresponsive to conventional systemic therapy. I As per USHSF 2019 guidelines: https://web.pathway.md/diseases/recLhlEqiLh0BxVCU 4/8 6/24/23, 12:45 PM Hidradenitis suppurativa Pathway Consider administering short-term pulse corticosteroid therapy in patients with acute flares or to bridge patients to other treatment. C Consider administering long-term systemic corticosteroids tapered to the lowest possible dose as adjunct therapy in patients with severe hidradenitis suppurativa with suboptimal response to standard therapy. Consider administering as low-dose adjunctive therapy or pulse dosing for acute flares or to bridge to other treatment. C Other immunosuppressants: As per BAD 2019 guidelines, insufficient evidence to support the use of the following immunosuppressants in patients with hidradenitis suppurativa unresponsive to conventional systemic therapy: azathioprine ciclosporin methotrexate colchicine. I As per USHSF 2019 guidelines, consider administering colchicine in combination with minocycline in patients with refractory mild-to-moderate hidradenitis suppurativa. C Show 2 more Biologic agents: As per BAD 2019 guidelines, offer adalimumab 40 mg weekly in patients with moderate-to- severe hidradenitis suppurativa unresponsive to conventional systemic therapy. A Show 3 more As per USHSF 2019 guidelines, offer adalimumab to improve disease severity and quality of life in patients with moderate-to-severe hidradenitis suppuratova. A Show 4 more Agents with no evidence for benefit: Insufficient evidence to support the use of the following agents in patients with hidradenitis suppurativa unresponsive to conventional systemic therapy: staphage lysate atorvastatin tolmetin sodium fumaric acid esters. I Pain management: achieve disease control as the first-line method for pain management in patients with hidradenitis suppurativa. B Show 2 more 4. Nonpharmacologic interventions Lifestyle modifications: As per BAD 2019 guidelines, refer patients with hidradenitis suppurativa to smoking cessation and weight management services, if relevant. E https://web.pathway.md/diseases/recLhlEqiLh0BxVCU 5/8 6/24/23, 12:45 PM Hidradenitis suppurativa Pathway As per USHSF 2019 guidelines, advise smoking cessation. B Show 2 more Supplements: As per BAD 2019 guidelines, insufficient evidence to support the use of oral zinc in patients with hidradenitis suppurativa unresponsive to conventional systemic therapy. I As per USHSF 2019 guidelines, consider offering oral zinc supplements. C 5. Therapeutic procedures Intralesional corticosteroids: As per BAD 2019 guidelines, consider administering intralesional corticosteroid injections in carefully selected hidradenitis suppurativa lesions during the acute phase. C As per USHSF 2019 guidelines, consider administering intralesional corticosteroid injections in the inflamed lesions for short-term control of hidradenitis suppurativa flares. Laser and light therapies: As per BAD 2019 guidelines, insufficient evidence to recommend the following therapies in patients with hidradenitis suppurativa unresponsive to conventional systemic therapy: laser therapy photodynamic therapy phototherapy photochemotherapy. I As per USHSF 2019 guidelines, offer Nd-YAG laser for patients with Hurley stage I disease and Hurley stage II and III disease. B Show 3 more Therapies with no evidence for benefit: As per BAD 2019 guidelines, insufficient evidence to recommend the following therapies in patients with hidradenitis suppurativa unresponsive to conventional systemic therapy: external beam radiation therapy hyperbaric oxygen therapy. Show 2 more As per USHSF 2019 guidelines, recognize that external beam radiation therapy and photodynamic therapy have a limited role in the management of patients with hidradenitis suppurativa. B 6. Perioperative care Perioperative medical therapy: continue medical therapy in the perioperative period as it is likely to be beneficial and poses minimal risk of increased postoperative complications. B https://web.pathway.md/diseases/recLhlEqiLh0BxVCU 6/8 6/24/23, 12:45 PM Hidradenitis suppurativa Pathway 7. Surgical interventions Surgery: perform deroofing or excision for recurrent nodules and tunnels. B Show 2 more Wound care: ensure wound healing following surgery through secondary intention, primary closure, delayed primary closure, flaps, grafts, and/or skin substitutes. B Show 4 more 8. Specific circumstances Pregnant patients: Offer topical treatments, procedures, and safe systemic agents in pregnant patients with hidradenitis suppuratova. Avoid using retinoids, hormonal agents, most systemic antibiotics, and most immunosuppressive medications in pregnant patients with hidradenitis suppuratova. 9. Patient education Patient education: provide a patient information leaflet in all patients with hidradenitis suppurativa. E 10. Follow-up and surveillance Assessment of treatment response: measure treatment response using recognized instruments for pain and quality of life, including an inflammatory lesion count for patients on adalimumab therapy. E References 1. Ali Alikhan, Christopher Sayed, Afsaneh Alavi 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. Open 2. J R Ingram, F Collier, D Brown et al. British Association of Dermatologists guidelines for the management of hidradenitis suppurativa (acne inversa) 2018. Br J Dermatol. 2019 May;180 5 1009 1017. Open 3. 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. Open https://web.pathway.md/diseases/recLhlEqiLh0BxVCU 7/8 6/24/23, 12:45 PM Hidradenitis suppurativa Pathway 4. Saunte DML, Jemec GBE. Hidradenitis Suppurativa: Advances in Diagnosis and Treatment. JAMA. 2017 Nov 28;318 20 2019 2032. Open 5. Calao M, Wilson JL, Spelman L et al. Hidradenitis Suppurativa HS prevalence, demographics and management pathways in Australia: A population-based cross-sectional study. PLoS One. 2018 Jul 24;13 7):e0200683. Open 6. 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 1 118 122. Open 7. Napolitano M, Megna M, Timoshchuk EA et al. Hidradenitis suppurativa: from pathogenesis to diagnosis and treatment. Clin Cosmet Investig Dermatol. 2017 Apr 19;10 105 115. Open 8. Vinkel C, Thomsen SF, . Hidradenitis Suppurativa: Causes, Features, and Current Treatments. J Clin Aesthet Dermatol. 2018 Oct;11 10 17 23. Open https://web.pathway.md/diseases/recLhlEqiLh0BxVCU 8/8