Clinical Policy Title: Hidradenitis suppurativa surgery

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1 Clinical Policy Title: Hidradenitis suppurativa surgery Clinical Policy Number: Effective Date: July 1, 2016 Initial Review Date: April 27, 2016 Most Recent Review Date: March 6, 2018 Next Review Date: March 2019 Policy contains: Hidradenitis suppurativa. Hurley staging system. Skin conditions. Related policies: None. ABOUT THIS POLICY: Select Health of South Carolina has developed clinical policies to assist with making coverage determinations. Select Health of South Carolina s clinical policies are based on guidelines from established industry sources, such as the Centers for Medicare & Medicaid Services (CMS), state regulatory agencies, the American Medical Association (AMA), medical specialty professional societies, and peerreviewed professional literature. These clinical policies along with other sources, such as plan benefits and state and federal laws and regulatory requirements, including any state- or plan-specific definition of medically necessary, and the specific facts of the particular situation are considered by Select Health of South Carolina when making coverage determinations. In the event of conflict between this clinical policy and plan benefits and/or state or federal laws and/or regulatory requirements, the plan benefits and/or state and federal laws and/or regulatory requirements shall control. Select Health of South Carolina s clinical policies are for informational purposes only and not intended as medical advice or to direct treatment. Physicians and other health care providers are solely responsible for the treatment decisions for their patients. Select Health of South Carolina s clinical policies are reflective of evidence-based medicine at the time of review. As medical science evolves, Select Health of South Carolina will update its clinical policies as necessary. Select Health of South Carolina s clinical policies are not guarantees of payment. Coverage policy Select Health of South Carolina considers the use of surgery for hidradenitis suppurativa to be clinically proven and, therefore, medically necessary when: Member has a confirmed diagnosis of hidradenitis suppurativa from a dermatologist. Disease is refractory to antibiotic treatments, acne washes and medicine, and bleach baths of 5 10 minutes. Disease is refractory to conservative medical therapy, including, but is not limited to: Local hygiene and ordinary hygiene. Use of antiseptic and antiperspirant agents (e.g., 6.25 percent aluminum chloride hexahydrate in absolute ethanol). Application of warm compresses with sodium chloride solution or Burow solution. Cessation of cigarette smoking. Medical anti-inflammatory or antiandrogen therapy such as oral or topical antibiotics, intralesional triamcinolone, spironolactone, or finasteride. 1

2 Weight reduction in patients who are obese. Wearing of loose-fitting clothing. Laser hair removal (Gulliver, 2016; Jovanic, 2017; Zouboulis, 2015). Limitations: All other uses of surgery for hidradenitis suppurativa are not medically necessary. Alternative covered services: Primary care services (i.e., patient education) Specialty services (i.e., dermatologic service) Background Hidradenitis suppurativa is a chronic inflammatory skin condition characterized by recurrent painful lesions, boils, nodules and abscesses that rupture and lead to sinus tracts and scarring in flexural sites (e.g., axillae and groin). Flares of the disease include foul-smelling discharges and pain causing quality of life impairment. Diagnosis can be made if the condition occurs at least twice in six months. In a study of 47,690 U.S. patients, females had more than twice as high a prevalence than did males (137 versus 58 per 100,000). African Americans had about three time higher a prevalence than whites (296 versus 95), and the age with the highest prevalence (172) were those in their 30s (Garg, 2018). Hidradenitis suppurativa affects about one percent of the general population in Europe, with onset in early adulthood (prevalence among young adult females is about four percent). Onset at the time of puberty has led several authorities to cite hormonal changes (occlusion of the apocrine duct by a keratinous plug, and defects of the follicular epithelium) as an etiologic factor in the development of hidradenitis suppurativa. An estimated 30 to 40 percent of persons with the disease report a family history. Cormibidities include obesity, diabetes, insulin resistance, glucose tolerance, and hyperlipidemia (Scuderi, 2017). Cigarette smoking also has a strongly positive correlation to the development of the condition, along with friction from axillary adiposity, sweat, heat, stress, tight clothing, and genetic and hormonal components (Shah, 2005). The oldest, and simplest, system for classification of hidradenitis suppurativa is the Hurley staging system. Hurley stage I is a single lesion without sinus tract formation. Stage II manifests as more than one lesion or area, but with limited tunneling. Stage III is defined as multiple lesions, with more extensive sinus tracts and scarring (Scuderi, 2017). Hidradenitis suppurativa is difficult to treat owing to its pervasive inflammation with abscesses and 2

3 inflammatory nodules, which leads to disruption of normal skin and subcutaneous architecture with sinus tract formation and, in severe cases, with extensive scarring. In contemporary practice, physicians often use the treatments below for hidradenitis suppurativa: Antibiotics can reduce inflammation, fight infection, prevent hidradenitis suppurativa from worsening, and stop new breakouts. Acne washes and medicines may be helpful, but these products alone usually will not clear hidradenitis suppurativa. Bleach baths of 5 or 10 minutes are also useful as adjunctive therapy. Other conservative treatments may include: Local hygiene and ordinary hygiene Weight reduction in patients who are obese Use of ordinary soaps and antiseptic and antiperspirant agents (e.g., 6.25% aluminum chloride hexahydrate in absolute ethanol) Application of warm compresses with sodium chloride solution or Burow solution Wearing of loose-fitting clothing Laser hair removal Cessation of cigarette smoking Medical anti-inflammatory or antiandrogen therapy such as oral or topical antibiotics, intralesional triamcinolone, spironolactone, or finasteride Biological therapy (Jovanivic, 2017). In refractory situations, a surgical procedure may be necessary: Incision and drainage, during which the surgeon drains one or two lesions or cuts them out. This can bring short-term relief, buthidradenitis suppurativa can return. Excision and primary closure (or deroofing) surgery may be an option for patients who have painful hidradenitis suppurativa that repeatedly returns. Radical excision involves surgically cutting out the hidradenitis suppurativa with a margin of normal-looking skin. Because the wound is deep, the area needs to be covered with a skin graft or a skin flap pulled from nearby skin to cover the wound. Surgical excision aims to effect complete eradication of involved skin and subcutaneous tissues, and avert any possibility of malignant change (i.e., squamous carcinoma); however, controversy surrounds the best procedure. Moderately severe axillary lesions can be treated adequately by excision and primary closure. This approach is particularly popular because it allows both axillae to be treated simultaneously in the many patients with bilateral involvement. Less invasive methods recently becoming more common include vacuum-assisted closure therapy, platelet-enriched plasma, and dermal substitutes. Surgery should only be limited to incision, drainage, and deroofing in the acute phase, while major surgery should only be performed in the silent chronic phase. Reconstruction after radical excision is important to maintain function, reduce contracture, and provide good aesthetic outcomes (Scuderi, 2017). 3

4 Based on the European Dermatology Forum guidelines (Gulliver, 2016) for the management of HS, all patients should be offered adjuvant therapy as needed (pain management, weight loss, tobacco cessation, treatment of super-infections, and application of appropriate dressings). The treating physician should be familiar with disease severity scores, especially Hurley staging. The need for surgical intervention should be assessed in patients with higher Hurley stages of disease. A European guideline recommends that locally recurring lesions can be treated by classical surgery or laser techniques; for widely spread lesions, medical treatment either as monotherapy or in combination with radical surgery is more appropriate (Zouboulis, 2015). Searches Select Health of South Carolina searched PubMed and the databases of: UK National Health Services Center for Reviews and Dissemination. Agency for Healthcare Research and Quality s National Guideline Clearinghouse and other evidence-based practice centers. The Centers for Medicare & Medicaid Services (CMS). We conducted searches on February 1, Search terms were: "hidradenitis suppurativa" and "surgery for hidradenitis suppurativa." We included: Systematic reviews, which pool results from multiple studies to achieve larger sample sizes and greater precision of effect estimation than in smaller primary studies. Systematic reviews use predetermined transparent methods to minimize bias, effectively treating the review as a scientific endeavor, and are thus rated highest in evidence-grading hierarchies. Guidelines based on systematic reviews. Economic analyses, such as cost-effectiveness, and benefit or utility studies (but not simple cost studies), reporting both costs and outcomes sometimes referred to as efficiency studies which also rank near the top of evidence hierarchies. Findings In a study of 251 patients with the disease, the median Hidradenitis Suppurativa Score was 41 for smokers, 27 for former smokers, and 22 for non-smokers. Median scores were progressively higher with increasing body mass index (32 for those with <25 kg, 44 for those with kg, and 50 for those with >30 kg (Sartorium, 2009). A study of 846 persons with hidradenitis suppurativa documented 45.5, 41.5, and 13.0 percent were considered to be Hurley stage I, II, and III, respectively. Severity was associated with male sex (p <.001), disease duration (p <.001), body mass index (p =.01), smoking pack-years (p =.001), and axillary (p <.001), perianal (p <.001), and mammary lesions (p =.03) (Schrader, 2014). Patients classified as severe 4

5 stage II or stage III were found to be more likely to benefit from surgery (Ellis, 2012), upholding the opinion of experts that surgery is mandatory in moderate and severe cases (Janse, 2016). A 2016 Cochrane review of 12 trials covering 15 treatment modes to treat hidradenitis suppurativa, found no randomized controlled trials on timing of surgery or type of surgical procedure (Ingram, 2016). A systematic review of various treatments of hidradenitis suppurativa, including excisional surgery, produced only four (of 62) studies that met criteria for strong scientific evidence (Rambhatla, 2012). A study of surgical treatment of chronic, severe hidradenitis suppurativa through wide excision technique and healing by secondary intention included17 patients underwent 23 separate surgical encounters, five with excision of multiple areas. Two patients developed recurrence adjacent to the surgical site (one requiring re-excision and the other treated with topical therapy), whereas two developed HS flares at distant nonsurgical sites managed medically. Authors concluded that wide excision of disease and healing by secondary intent demonstrated clinically satisfactory functional and excellent aesthetic results in multiple anatomic areas and even for large defects. They warned, however, that healing by secondary intention requires strict adherence to a wound-care protocol (Humphries, 2016). A review of 118 surgical procedures to 57 patients with hidradenitis suppurativa observed 44 patients (77.2 percent) showed good tolerance of the operation and during the postoperative period, compared to only one (1.8) reporting unsatisfactory tolerance. A total of 51 (89.5) expressed willingness to undergo additional surgery in the event of lesion recurrence. Complete recovery was observed in 34 (59.7) and partial recovery in 18 (31.6), and no improvement in five (8.8) after two years, leading authors to conclude that surgery is effective and well tolerated (Beniek, 2010). There is contemporary interest in a combined biologic and surgical approach to therapy for recalcitrant hidradenitis suppurativa. A study of combined therapy between 2011 and 2014 (DeFazio, 2015) described 21 patients (57 cases) with Hurley Stage III hidradenitis suppurativa who underwent radical resection with delayed primary closure alone, or in combination with adjuvant biologic therapy. Eleven patients underwent combined surgical and biologic therapy, and radical resection alone was performed in 10 patients. The average soft tissue deficit, before closure, for the combined and surgery-only patients was 56 cm and 48.5 cm, respectively (p = 0.66). Biologic agents including infliximab (n = 8) and ustekinumab (n = 3) were initiated two to three weeks after closure and were continued for an average of 10.5 months. Recurrence was noted in 19 percent (4/29) and 38.5 percent (10/26) of previously treated sites for combined and surgery-only patients (p < 0.01). For the combined cohort, the disease-free interval was approximately one year longer on average (p < 0.001); however, this difference was reduced to 4.5 months when considering time to recurrence after cessation of biologic therapy (p = 0.09). New disease developed in 18 percent (2/11) and 50 percent (5/10) of combined and surgery-only patients, respectively (p < 001). No adverse events were noted among patients who received biologic therapy. The authors concluded that lower rates of recurrence and disease progression, as well as a longer disease-free interval, may be achieved with the use of adjuvant biologic therapy after radical resection for recalcitrant hidradenitis suppurativa. 5

6 Recurrence was the topic of a systematic review and meta-analysis of 22 articles. The percent of cases with a documented recurrence included 13.0 percent for wide incision, 22.0 percent for local incision, and 27.0 percent for deroofing. Subjects in the wide excision group had a much lower recurrence rate using flaps and grafting (eight and 6 percent), compared to 15 percent for flaps (Mehdizadeh, 2015). A study of 200 patients who underwent surgery for hidradenitis suppurativa included 76 with surgical excision with primary closure, and 124 with primary closure over a gentamicin-collagen sponge. Significantly fewer complications such as infection and dehiscence were noted in the sponge group (35 versus 52 percent) after one week; after three months, complication and recurrence rates in the groups were comparable, at 12 versus 19 percent, and 40 versus 42 percent (Buimer, 2008). Prevention of adverse effects is an aspect of hidradenitis suppurativa that has generally been ignored. A study comparing both designs included 7,732 patients with 4,354,137 matched controls, the suicide rate was significantly higher for patients (p =.00334), based on 11 suicides. Among patients, antidepressant drug use was significantly greater (p <.0001), and insignificantly greater for depression (p = 0.36) and hospitalization due to depression (p < 0.11) (Thorlacius, 2018). Another study of prevention for 50 procedures on 32 patients with hidradenitis suppurativa indicated that antibiotics and minor surgery, then stabilizing the patient before wide surgical excision reduced recurrence (only six of 32) and complications (Alharbi, 2012). A utilization study divided 150,493 persons into those with 1) at least two claims for hidradenitis suppurativa, 2) at least two claims for psoriasis, and 3) no claims for either condition over a three-year period. The proportion with at least one hospitalization (for any condition) for the hidradenitis suppurativa, psoriasis, and control groups were 15.8, 10.8, and 8.6 percent. Corresponding percentages of how many in each group used the emergency department were 27.1, 17.4, and 17.2 (Kirby, 2014). Policy updates: A total of two guidelines/other and 13 peer-reviewed references were added to, and four peer-reviewed references removed from, this policy in February Summary of clinical evidence: Citation Humphries (2016) Content, Methods, Recommendations Key points: Wide excision and healing by secondary intent for the surgical treatment of HS: a single-center experience. This study reviewed a single center's 14-year experience with surgical treatment of chronic, severe HS through wide excision technique and healing by secondary intention. All patients who underwent wide excision of HS between 2000 and 2014 and allowed to heal by secondary intention were included. Wound care consisted of topical antimicrobials and hydrotherapy. Physical therapy was initiated for joint contracture 6

7 Citation DeFazio (2015) Outcomes after combined radical resection and targeted biologic therapy for the management of recalcitrant HS. Kirby (2014) Content, Methods, Recommendations prevention. Patients were followed until complete wound closure. Seventeen patients underwent 23 separate surgical encounters, five with excision of multiple areas. Seventeen excisional procedures were conducted on the upper half of the body (axillary and breast) and 11 on the lower half (inguinal, perineum, perianus and abdomen). Two patients developed HS recurrence adjacent to the surgical site (one requiring reexcision and the other treated with topical therapy), whereas two developed HS flares at distant nonsurgical sites managed medically. The mean follow-up was 1.02 years with a median of six months, ranging from 1.2 months to 5.25 years. Complete wound healing ranged from 8 weeks to 16 months, with limited ROM in two patients. Key points: Between 2011 and 2014, 21 patients (57 cases) with Hurley Stage III HS underwent radical resection with delayed primary closure alone, or in combination with adjuvant biologic therapy. Eleven patients underwent combined surgical and biologic therapy, whereas radical resection alone was performed in 10 patients. The average soft tissue deficit, before closure, for the combined and surgery-only patients was 56 cm and 48.5 cm, respectively (P = 0.66). Biologic agents including infliximab (n = 8) and ustekinumab (n = 3) were initiated two to three weeks after closure and were continued for an average of 10.5 months. Recurrence was noted in 19 percent (4/29) and 38.5 percent (10/26) of previously treated sites for combined and surgery-only patients (P < 0.01). For the combined cohort, the disease-free interval was approximately one year longer on average (P < 0.001); however, this difference was reduced to 4.5 months when considering time to recurrence after cessation of biologic therapy (P = 0.09). New disease developed in 18 percent (2/11) and 50 percent (5/10) of combined and surgery-only patients, respectively (P < 001). No adverse events were noted among patients who received biologic therapy. Lower rates of recurrence and disease progression, as well as a longer disease-free interval may be achieved with the use of adjuvant biologic therapy after radical resection for recalcitrant HS. Key points: Health care utilization patterns and costs for patients with HS. A cohort cost-identification study of 16,736 individuals with claims for HS measured inpatient length of stay, emergency department and outpatient visits, and number of days supplied of prescription medication. The largest component of the total three-year cost for the HS group was inpatient cost (37.4 percent). The proportion of people who were hospitalized in the HS cohort (15.8 percent) was higher than control (8.6 percent) groups (P <.001). The proportion of patients who sought emergency room (ER) care over the three-year period was higher in the HS cohort (27.1 percent) than in the control group (17.2 percent) (P <.001), with the mean three-year ER cost for the HS group of $2,002. 7

8 Citation Beniek (2010) Content, Methods, Recommendations Key points: Outcomes of surgery for hidradenitis suppurativa Review of 118 surgical procedures to 57 patients with hidradenitis suppurativa. 44 patients (77.2%) showed good tolerance of the operation and during the postoperative period; one patient (1.8%) reporting unsatisfactory tolerance. 51 patients (89.5%) willing to undergo additional surgery if lesion recurred. Complete recovery was observed in 34 (59.7%), partial recovery in 18 patients (31.6%), no improvement in five patients (8.8%) after two years Authors conclude that surgery is effective and well tolerated References Professional society guidelines/other: Gulliver W, Zouboulis CC, Prens E, Jemec GB, Tzellos T. Evidence-based approach to the treatment of hidradenitis suppurativa/acne inversa, based on the European guidelines for hidradenitis suppurativa. Rev Endocr Metab Disord. 2016;17(3): Jovanovic M. Hidradenitis suppurativa treatment & management. Medscape, updated August 7, Accessed January 31, 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;29(4): Peer-reviewed references: Alharbi Z, Kauczok J, Pallua N. A review of wide surgical excision of hidradenitis suppurativa. BMC Dermatol. 2012;12:9. Beniek A, Matusiak L, Okulewicz-Gojlik D, Szepietowski JC. Surgical treatment of hidradenitis suppurativa: experiences and recommendations. Dermatol Surg. 2010;36(12): Buimer MG, Ankersmit MF, Wobbes T, Klinkenbijl JH. Surgical treatment of hidradenitis suppurativa with gentamicin sulfate: a prospective randomized study. Dermatol Surg. 2008;34(2): DeFazio MV, Economides JM, King KS, et al. Outcomes after combined radical resection and targeted biologic therapy for the management of recalcitrant hidradenitis suppurativa. Ann Plast Surg. 2016;77(2): Ellis LZ. Hidradenitis suppurativa: surgical and other management techniques. Dermatol Surg. 2012;38(4):

9 Garg A, Kirby JS, Lavian J, Lin G, Strunk A. Sex- and age-adjusted population analysis of prevalence estimates for hidradenitis suppurativa in the United States. JAMA Dermatol. 2017;153(8): Hamzavi IH, Griffith JL, Riyaz F, Hessam S, Bechara FG. Laser and light-based treatment options for hidradenitis suppurativa. Dermatol. 2015; 73(5 Suppl 1): S Humphries LS, Kueberuwa E, Beederman M, Gottlieb LJ. Wide excision and healing by secondary intent for the surgical treatment of hidradenitis suppurativa: A single-center experience. Rev Endocr Metab Disord. 2016;69(4): Ingram JR, Woo PN, Chua SL, et al. Interventions for hidradenitis suppurativa: a Cochrane systematic review incorporating GRADE assessment of evidence quality. Br J Dermatol. 2016;174(5): Janse I, Bieniek A, Horváth B, Matusiak Ł.Surgical procedures in hidradenitis suppurativa. J Am Acad Dermatol Clin. 2016; 34(1): Kirby JS, Miller JJ, Adams DR, Leslie D. Health care utilization patterns and costs for patients with hidradenitis suppurativa. JAMA Dermatol. 2014; 150(9): Mehdizadeh A, Hazen PG, Bechara FG, et al. Recurrence of hidradenitis suppurativa after surgical management: A systematic review and meta-analysis. J Am Acad Dermatol. 2015;73(5 Suppl 1):S Rambhatla PV, Lim HW, Hamzavi I. A systematic review of treatments for hidradenitis suppurativa. Arch Dermatol. 2012;148(4): Sartorius K, Emtestam L, Jemec GB, Lapins J. Objective scoring of hidradenitis suppurativa reflecting the role of tobacco smoking and obesity. Br J Dermatol. 2009;161(4): Schrader AM, Deckers IE, van der Zee HH, Boer J, Prens EP. Hidradenitis suppurativa: a retrospective study of 846 Dutch patients to identify factors associated with disease severity. J Am Acad Dermatol. 2014;71(3): Scuderi N, Monfrecola A, Dessy LA, Fabbrocini G, Megna M, Monfrecola G. Medical and surgical treatment of hidradenitis suppurativa: a review. Skin Appendage Disord. 2017;3: Shah N. Hidradenitis suppurativa: a treatment challenge. Am Fam Physician. 2005;72(8): Thorlacius L, Cohen AD, Gisalason GH, Jemec GBE, Egeberg A. Increased suicide risk in patients with hidradenitis suppurativa. J Invest Dermatol. 2018;138(1): CMS National Coverage Determinations (NCDs): 9

10 No NCDs identified as of the writing of this policy. Local Coverage Determinations (LCDs): No LCDs identified as of the writing of this policy. Commonly submitted codes Below are the most commonly submitted codes for the service(s)/item(s) subject to this policy. This is not an exhaustive list of codes. Providers are expected to consult the appropriate coding manuals and bill in accordance with those manuals. CPT Code Description Comment Incision and drainage of abscess (eg, carbuncle, suppurative hidradenitis, cutaneous or subcutaneous abscess, cyst, furuncle, or paronychia); simple or single Excision of skin and subcutaneous tissue for hidradenitis, axillary; with simple or intermediate repair Excision of skin and subcutaneous tissue for hidradenitis, axillary; with complex repair Excision of skin and subcutaneous tissue for hidradenitis, inguinal; with simple or intermediate repair Excision of skin and subcutaneous tissue for hidradenitis, inguinal; with complex repair Excision of skin and subcutaneous tissue for hidradenitis, perianal, perineal, or umbilical; with simple or intermediate repair Excision of skin and subcutaneous tissue for hidradenitis, perianal, perineal, or umbilical; with complex repair ICD-10 Code Description Comment L73.2 Hidradenitis suppurativa HCPCS Level II Code N/A Description Comment 10