Hidradenitis Suppurativa. Young-San, Jeon. Department of Surgery Thyroid and breast center, Goo Hospital

Transcription

1 Hidradenitis Suppurativa Young-San, Jeon Department of Surgery Thyroid and breast center, Goo Hospital

2 Definition Hidradenitis suppurativa/acne inversa (HS) is a chronic, inflammatory, recurrent, debilitating skin disease of the hair follicle Historical background First described in 1839 by Velpeau In 1854, French Surgeon Verneuil gave a name to this disorder Common Site Axilla Groin Buttock Inframammry or intermammary fold Concomittent ds. Inflammatory bowel ds. Hypothyroidism

3 Definition

4 Epidemiology Incidence average global prevalence of 1% : not rare F:M = 2~5:1 but axilla : no gender prediction?? Mean age of onset : 22.1yrs The average interval from the onset of symptoms to diagnosis is 7.2 years References: 1. Sundaram M, et al. EADV. 2012:P von der Werth. JEADV. 2000;14: Cosmatos, et al. JAAD. 2013;68(3): Jemec GB. N Engl J Med. 2012;366(2): Palmer R, Keefe M. Clin Dermatol. 2001;26:

5 Pathogenesis The exact cause of HS is unknown, inappropriated immune response(hypothesis)

6 Risk Factor Associated Smoking HS vs matched controls: OR = Correlated with HS severity Obesity HS vs matched controls: OR = 4.42 Correlated with HS severity Female sex Female:male ratio, 3:1 Genetic 35~40% family history Causal Friction/mechanical Medications (eg, lithium) Hormonal influences(sex hormone) Female predominance HS declines after menopause HS can flare premenses improves with pregnancy No evidence of hyperandrogenism in most studies References: 1. Jemec GB. N Engl J Med. 2012;366(2): Revuz JE, et al. J Am Acad Dermatol. 2008;59(4): Alikhan A, et al. J Am Acad Dermatol. 2009;60(4): Yazdanyar S, et al. Curr Opin Infect Dis. 2011;24(2):

7 Clinically!!! Based diagnosis Diagnosis Most laboratory investigations and Bx. are unhelpful Primary positive diagnostic criteria History: Recurrent painful or suppurating lesions more than 2 /6 months Typical site : axilla, bottock, genitofemoral area, infra or inter mammary area Typical lesion : Presence of nodules (inflamed or noninflamed) sinus tracts (inflamed or noninflamed) abscesses scarring (atrophic, red, hypertrophic or linear)

8 Diagnosis Typical lesion Nodules(inflamed noninflamed) Fistula(inflamed or noninflamed),scarring (hypertrophic or linear), Abscess orifice Fistula Sinus Tract

9 Diagnosis Seven questions which aid in the diagnosis of HS: Yes supports HS diagnosis 1. Family with same symptoms? 2. Boils recur in same spot? 3. Smoke tobacco? 4. Premenstrual flares? No supports HS diagnosis 1. Previous treatment helped? 2. Infections elsewhere? 3. Fever with boils?

10 Severity Assessment - Hurley Stage Hurley Stage I II III Description Abscess formation, single or multiple, without sinus tracts and cicatrization Single or multiple, widely separated, recurrent abscesses with tract formation and cicatrization Diffuse or near-diffuse involvement, or multiple interconnected tracts and abscesses across the entire area

11 Severity Assessment - PGA Description HS-PGA ( Hidradenitis Suppurativa Physician s Global Assessment ) Clear (0) Minimal (1) Mild (2) Moderate (3) Severe (4) Very severe (5) 0 abscesses, 0 draining fistulas, 0 inflammatory nodules, and 0 noninflammatory nodules 0 abscesses, 0 draining fistulas, 0 inflammatory nodules, and presence of noninflammatory nodules 0 abscesses, 0 draining fistulas, and 1 4 inflammatory nodules; or 1 abscess or draining fistula and inflammatory nodules 0 abscesses, 0 draining fistulas, and 5 inflammatory nodules; or 1 abscess or draining fistula and 1 inflammatory nodule; or 2 5 abscesses or draining fistulas and <10 inflammatory nodules 2 5 abscesses or draining fistulas and 10 inflammatory nodules >5 abscesses or draining fistulas Reference: 1. Kimball A, et al. BJD. 2014;171:

12 Treatment for HS 1. Life-style modification 2. Antibiotics 3. Biologics 4. Surgery 5. Others.. There is no single effective treatment for HS Combination of available treatments

13 Treatment for HS Life-style modification Reduce heat, sweating and friction in the area Loose clothing, boxer-type underwear Stop all dairy products Low glycemic load diet Stop smoking! Reduction of Weight!

14 Treatment guideline for HS Therapy 1st Line Category of Evidence Strength of Recommendation Clindamycin (topical) IIb Possible B Clindamycin/Rifampicin (oral) III C Adalimumab (SC) Ib A Tetracycline (oral) IIb B Surgery Excision (individual lesions) III C Total excision (lesions and surrounding hair-bearing skin) Second Intention Healing IIb B Primary Closure III C Reconstruction with Skin Grafting and NPWT Reconstruction with Flap Plasty Ia/IIa A/B Deroofing IV D Carbon Dioxide Laser Therapy Ib A Nd:YAG Laser Ib A IPL IV D IIb III B C 2nd Line Zinc Gluconate III C Resorcinol III C Intralesional Steroids IV D Systemic Corticosteroids IV D Infliximab Ib/Ia B Acitretin/Etretinate III C 3rd Line Clochicine IV D Botulinum Toxin IV D Dapsone IV D Cyclosporine IV D Hormones IV D Pain Control NSAIDS IV D Opiates IV D Dressings No studies IV D References: 1. Gulliver et al. Rev Endocr Metab Disord Feb 1. [Epub ahead of print]. PMID:

15 Treatment for HS - Surgery Radical Excision of all involved tissue, beyond clinically involved margins with 1-2cm, is the most effective treatment modality

16 Treatment guideline for HS

17 Treatment guideline for HS Medical treatment Surgical treatment Hurley I and mild II Hurley II and III First line Clindamycin topical x 2 for 12 weeks Tetracyclin systemic 500 mg x 2, up to 4 months Tetracyclin systemic 500 mg x 2, up to 4 months Clindamycin 300 mg x 2 plus Rifampicin 600 mg up to 10 weeks Adalimumab 40 mg /week (160 mg 0 week, 80 mg week2, 40 mg/week from week 4) Second line Zinc gluconate Resorcinol Intralesional triamcinolon Hormones Cyclosporine Infliximab Acitretin/Etretinate Alitretinoin Third line Colchicine Botulinum toxin Dapsone Local excision De-roofing Intense pulse light Nd:YAG laser wide excision

18 Biologic Tx. for HS - Adalimumab Significant elevations in levels of the cytokines IL-1β, IL-10, tumor necrosis factor α (TNF-α) detected in HS lesions. Adalimumab, IgG1 monoclonal antibody for TNF-α PIONEER I and II Study Multicenter, double blined, well cross-matched

19 moderte to severe HS (Hurley stage II, III) and inadequate response to oral antibiotics Biologic Tx. for HS - Adalimumab PIONEER I Study PIONEER II Study

20 Biologic Tx. for HS - Adalimumab HUMIRA (adalimumab 40mg) : 414,850 원 ( 보험가 ) 월부터급여 (HS) (160 mg 0 week, 80 mg week2, 40 mg/week from week 4) 투여대상다음가 ), 나 ), 다 ) 조건을동시에충족하는경우 - 다음 - 가 ) 화농성한선염최초진단후 1 년이상경과한 18 세이상성인으로, 나 ) 2 개이상의각기다른부위에병변이있고, 농양과염증성결절수의합이 3 개이상이며, 다 ) 항생제로 3 개월이상치료하였으나치료효과가미흡하거나부작용등으로치료를중단한중증 (Hurley stage III) 환자 평가방법동약제를 12 주간사용후농양 (abscess) 또는배출누관 (draining-fistula) 개수의증가가없으며, 농양과염증성결절수의합 (total abscess and inflammatory- nodule count) 이 50% 이상감소한경우추가 24 주간의사용을인정함.

21 Case I 27 세 /F 2017 년 12 월 exam : Hurley stage I disease duration : 2011 년부터피지라는이야기듣고 hospital tour ( 4 군데 ) dressing and antibiotic Tx. 본원내원 drainage procedure + Clindamycin 300 mg x 2 plus Rifampicin 600 mg qd. for 2months VS

22 Case II 27 세 /F 2018 년 1 월 Both axilla recurrent abscess for 3yrs Local 에서 antibiotic Tx. 본원내원 Rt. Drainage procedure, Lt. excision Humira 2 달째 지금치료중 Lt. Rt.

23 Case III 42 세 /M C/C 약 10 년동안의 both axillary pain and recurrent abscess (2014/01) Local 에서약 7 번수술본원에서 Lt. axilla and Rt. Axilla multiple I and D (2014/01) Lt. axilla radical excision ( 2015/07) 2017/11

24 Case III 2017/11

25 Case III Clindamycin 300 mg x 2 plus Rifampicin 600 mg up to 8 weeks Adalimumab 40 mg /week (160 mg 0 week, 80 mg week2, 40 mg/week from week 4) - 현재까지 injection 중 2017/ / /02

26 Case IV 17 세 /M C/C Lt. axillary painful nodule for 2 months Local 에서 Deroofing 1 차례이후 redness with pain 이심했다가덜했다가반복 FHx. : 아버지가 both buttock 에약 10 년간 multiple abscess 로 local 에서 antibiotic, I and D

27 Massage!! 피부과? 성형외과? 유방외과? 피부과? 성형외과? 대장항문외과?

28 Thank you! For Your Attention