Guideline Summaries
US Hidradenitis Suppurativa Foundation and the Canadian Hidradenitis Suppurativa Foundation guidelines
Guidelines on the diagnosis and evaluation; complementary and procedural management; and topical, intralesional, and systemic medical management of hidradenitis suppurativa were released in July 2019 by the US Hidradenitis Suppurativa Foundation and the Canadian Hidradenitis Suppurativa Foundation. [100, 101]
Grading and classification
Recommended grading tools are clinical performance, Hurley staging, and abscess and inflammatory lesion counts. The Visual Analogue Scale (VAS) and the Dermatology Life Quality Index (DLQI) also can be considered. For research studies, the recommended grading systems are the Hidradenitis Suppurativa Clinical Response (HiSCR) score, Hidradenitis Suppurativa Physician Global Assessment (HS-PGA), Sartorius score, DLQI, and pain VAS. Others to consider are the Hidradenitis Suppurativa Impact Assessment (HSIA) and the Hidradenitis Suppurativa Symptom Assessment (HSSA).
Screening for comorbidities
The patient should undergo a physical examination and a review of systems, with screening for metabolic syndrome, diabetes, depression, anxiety, polycystic ovarian syndrome, and tobacco abuse. If patients have additional risk factors for diabetes (eg, obesity, hyperlipidemia, hypertension, acanthosis nigricans), refer them for HbA1c and/or fasting glucose testing. [102] Based on the review of systems, other screening considerations are depression, inflammatory bowel disease, autoinflammatory syndromes, and inflammatory arthropathy.
Lifestyle modifications and alternative treatments
Patients should be counseled to quit smoking. If the patient is obese, weight loss should be recommended. As far as alternative treatments, zinc supplementation may be recommended but the evidence is weak. Vitamin D supplementation lacks sufficient evidence, as does avoidance of dairy or brewer's yeast, friction, deodorant, and shaving/depilation.
Surgical modalities
Deroofing or excision is recommended for recurrent nodules and tunnels. Incision and drainage should only be used for pain relief from acute abscesses. For extensive chronic lesions, use wide local scalpel, carbon dioxide, or electrosurgical excision (with or without reconstruction). Secondary intention healing, primary closure, delayed primary closure, flaps, grafts, or skin substitutes are all appropriate for wound healing. It is likely beneficial to continue medical treatment during the perioperative period; this poses minimal risk for increased postoperative complications.
Pain management
Disease control is paramount for pain management. Pain management involves considering the multidimensional aspects of pain. Short-acting opioids may be needed in select cases; dosing should be individualized and drugs carefully prescribed. Use of the World Health Organization pain ladder is recommended for chronic pain management.
Wound care
Follow the principals of best-practice individualized wound care for local wound care in surgical and nonsurgical wounds. Consider periwound skin condition, location, amount of drainage, cost, and patient preference when choosing the type of dressing. While it carries a low risk of contact dermatitis, use of antiseptic washes is generally supported by expert opinion. Negative-pressure therapy may be beneficial for selected large open wounds (for 1-4 wk), followed by delayed reconstruction.
Light, laser, and energy sources
Nd:YAG laser is recommended in those with Hurley stage I disease based on expert consensus. It is also recommended in those with Hurley stage II or III disease based on randomized controlled trial and case series data. Lower quality evidence suggests other wavelengths that are used for follicular destruction may be helpful. In patients with Hurley stage II or III disease with fibrotic sinus tracts, carbon dioxide laser is recommended. Photodynamic therapy and external beam radiation have limited roles.
Topical and intralesional therapies
Topical clindamycin can be used to reduce pustules; however, it carries a high risk of bacterial resistance. Resorcinol 15% cream is recommended; however, it may induce contact dermatitis. Expert opinion supports using antibacterial washes such as chlorhexidine, zinc pyrithione, or others. Intralesional corticosteroid injections can be considered for short-term control of inflamed lesions, but the evidence for this recommendation is weak.
Systemic antibiotics
For mild-to-moderate disease, tetracyclines are recommended for a 12-week course or for long-term maintenance when appropriate. For mild-to-moderate disease or for a first-line or adjunctive treatment in severe disease, combination therapy with clindamycin and rifampin is an effective second-line treatment. For moderate-to-severe disease, combination therapy with metronidazole, moxifloxacin, and rifampin is recommended as second- or third-line treatment. A minority of patients with Hurley stage I or II disease may benefit from dapsone as long-term maintenance therapy. For severe disease as a one-time rescue treatment or a bridge to surgery or other long-term maintenance, intravenous ertapenem is recommended. Balance the benefit achieved for each patient against the antibiotic resistance risk when determining the frequency and duration of antibiotic use. Disease recurrence is common following cessation of antibiotic therapy.
Hormonal agents
All described evidence for hormonal therapies has major limitations, based on variable outcome measures and methods, small samples sizes, and reporting bias. Estrogen-containing combined oral contraceptives, cyproterone acetate, spironolactone, finasteride, and metformin can be considered in appropriate female patients. These can be used as monotherapy for mild-to-moderate disease or in combination with other agents for more severe disease. Progestogen-only contraceptives should likely be avoided, as anecdotal data suggest they may worsen hidradenitis suppurativa.
Retinoids
Owing to mixed results from isotretinoin studies, it should be considered only as a second- or third-line treatment or in patients with severe concomitant acne. Acitretin should also be considered a second- or third-line treatment; it may be superior to isotretinoin, but significantly robust comparative studies are lacking. While not available in the United States, alitretinoin is supported by a single study in women. It is available in Canada and other countries.
Immunosuppressants
Based on the available limited evidence, methotrexate or azathioprine is not recommended. While the evidence is weak, combination colchicine/minocycline can be considered for refractory mild-to-moderate disease; avoid colchicine monotherapy. In patients with recalcitrant moderate-to-severe disease in whom standard therapies have failed or who are not candidates for standard therapy, consider cyclosporine. In acute flares or as a bridge to other treatments, short-term pulse steroid therapy can be considered. In cases of severe disease, consider using long-term corticosteroids, tapered to the lowest dose, as adjunctive therapy when the response to standard therapy has been suboptimal.
Biologics
Adalimumab and infliximab are recommended for moderate-to-severe disease. Adalimumab should be administered at the dosage approved for hidradenitis suppurativa. Dose-ranging studies are needed to determine the optimal dosage for infliximab. Agents that may be effective include anakinra (100 mg daily) and ustekinumab (45-90 mg q12wk). Dose-ranging studies are needed for anakinra, and placebo-controlled dose-ranging studies are needed for ustekinumab. Etanercept use is not supported by the limited available evidence.
Pediatric and pregnant patients
For pediatric patients with hidradenitis suppurativa, a laboratory evaluation for precocious puberty should be performed in those aged 11 years or younger when other suggestive physical examination findings are present. Additionally, avoid tetracyclines in children younger than 9 years. Avoid administration of acitretin to female patients during childbearing years. Agents to be avoided by pregnant patients with hidradenitis suppurativa include hormonal agents, retinoids, most immunosuppressive medications, and most systemic antibiotics. Topical treatments, procedures, and safe systemic agents are acceptable for use in pregnant patients.
European Dermatology Forum guidelines
In the published guidelines for hidradenitis suppurativa developed by the Guidelines Subcommittee of the European Dermatology Forum, [1] it is recommended that hidradenitis suppurativa be treated based on the subjective impact and objective severity of the disease, as follows:
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Locally recurring lesions can be treated surgically
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Medical treatment either as monotherapy or in combination with surgery is more appropriate for widely spread lesions
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Medical therapy may include antibiotics and immunosuppressants
A Hurley severity grade‒relevant treatment of hidradenitis suppurativa is recommended by the expert group with the following treatment algorithm:
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Limited surgery such as deroofing and laser ablation techniques are especially suited for recurrent hidradenitis suppurativa lesions at fixed locations in Hurley 1/mild Hurley II stage
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Wide surgical excision is appropriate for moderate Hurley II/Hurley III stage
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Topical clindamycin is recommended for localized Hurley I stage
Systemic treatment (clindamycin + rifampin/tetracycline or acitretin) with adjuvant therapy (pain management treatment of superinfections is proposed for Hurley II stage)
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Systemic biologics (adalimumab/infliximab) are reserved for treatment-resistant, moderate-to-severe hidradenitis suppurativa (moderate Hurley II/Hurley III stage)
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General measures are offered for all patients and include weight loss and tobacco abstinence [1]
Based on expert opinion it is recommended that adjuvant therapy is offered to all patients in the form of general measures such as weight reduction, cessation of cigarette smoking, and specific help with bandaging lesions in order to improve the patients’ quality of life. Hidradenitis suppurativa‒specific bandages are not currently available. [1] Choice of dressing is based on clinical experience. [54] In addition, adhesive tape should be avoided to minimize trauma to inflamed skin, which can be overcome by using tubular net bandages or superabsorbent pads or materials in the seams of clothing. [2]
Regarding local wound care, superabsorbent dressings are best to treat actively draining lesions or postoperative wounds, but there are no trials or studies to support this recommendation. [1] In order to prevent the primary dressing from sticking to the wound, white petrolatum, zinc oxide paste, or film-forming liquid acrylate should be extensively and generously applied on the marginal skin as the best ways to keep the wound dressings in place. [2]
Algorithm
In 2016, an algorithm with respect to all aspects of hidradenitis suppurativa therapy included in the aforementioned guidelines was developed by using Grading of Recommendations Assessment and Evaluation (GRADE) methodology based on the Category of Evidence and Strength of Recommendation. [1, 54]
The need for surgical intervention should be assessed in all patients depending on the type and extent of scarring. [54]
The proposed dosing regimen as a first-line treatment option in patients with mild hidradenitis suppurativa PGA or localized Hurley I/mild Hurley II stage, especially when there are no deep inflammatory lesions (abscesses), is topical clindamycin 1% solution/gel twice daily for 12 weeks and/or tetracycline 500 mg orally twice daily for 4 months.
If patient fails to exhibit a response to treatment or for a PGA of moderate-to-severe disease with moderate and severe hidradenitis suppurativa PGA or Hurley II stage, considered clindamycin 300 mg orally twice daily with rifampin 600 orally twice daily for 10 weeks.
If the patient is not improved, then adalimumab is recommended as a first-line treatment option in patients with moderate-to-severe hidradenitis suppurativa who were unresponsive to or intolerant of oral antibiotics. Dosing is adalimumab 160 mg at week 0, 80 mg at week 2, and then 40 mg subcutaneously weekly.
If improvement occurs, then therapy should be maintained as long as hidradenitis suppurativa lesions are present. If the patient fails to exhibit response, then consideration of second- or third-line therapy is required.
The second-line therapies include the following:
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Zinc gluconate
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Resorcinol
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Intralesional corticosteroids
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Infliximab at 5 mg/kg at week 0, 2, and 6, and then every 2 months thereafter for 12 weeks; recommended only after failure of adalimumab, in patients with moderate-to-severe hidradenitis suppurativa
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Acitretin
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Etretinate
If clinical response is not achieved after 12 weeks of treatment, other treatment modalities must be considered.
Third line therapies evaluated include the following:
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Colchicine
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Botulinum toxin
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Isotretinoin
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Dapsone
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Cyclosporine
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Hormones
Benefit-to-Risk Ratio
A relevant benefit-to-risk ratio analysis can be performed only for the phase 2 trial of adalimumab, since that is the only randomized controlled trial with an appropriate safety analysis that provides the basis to recommend adalimumab as the first-line treatment option in patients with moderate-to-severe hidradenitis suppurativa who were unresponsive to or intolerant of oral antibiotics. [54, 103]
There is very limited or absent randomized controlled trial data in hidradenitis suppurativa for antibiotic therapy, retinoids, oral immunomodulators, and, in particular, there are no randomized controlled trial data investigating the timing of surgery or type of surgical procedure. Interventions currently under investigation include topical antiseptics, the Nd:YAG and carbon dioxide lasers, anakinra (a newer biological treatment that inhibits IL-1, and the PIONEER I and II studies of adalimumab therapy. [103]
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Vulvar hidradenitis suppurativa.
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Vulvar and inguinal indurations.
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Sinus tract.
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Draining sinus tract.
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Axillary hidradenitis suppurativa in a patient with pyoderma gangrenosum.
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Close-up view of axillary hidradenitis suppurativa in a patient with pyoderma gangrenosum.
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Submammary hidradenitis suppurativa in a patient with pyoderma gangrenosum.
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Double-ended-comedones. Hidradenitis suppurativa in a patient with pyoderma gangrenosum.
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Inguinal hidradenitis suppurativa in a patient with pyoderma gangrenosum.
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Close-up view of inguinal hidradenitis suppurativa in a patient with pyoderma gangrenosum.
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Pyoderma gangrenosum in a patient with hidradenitis suppurativa.
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Close-up view of pyoderma gangrenosum in a patient with hidradenitis suppurativa.
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Coexisting hidradenitis suppurativa and pyoderma gangrenosum.
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Coexisting hidradenitis suppurativa and pyoderma gangrenosum.
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Hidradenitis suppurativa in a patient with pyoderma gangrenosum.
