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hidradenitis suppurativa; cicatrizing perifolliculitis; follicular occlusion syndrome; acne inversa

Abscesses of apocrine glands in the axillae, anogenital region & rarely scalp. Etiology: 1) unknown 2) predisposing factors a) obesity b) acne c) apocrine gland obstruction d) secondary bacterial infection e) stress f) cigarette smoking 3) may occur in association with: a) Crohn's disease b) irritable bowel syndrome c) Down syndrome d) arthritis e) Graves disease, Hashimoto thyroiditis f) Sjogren syndrome g) Herpes simplex Epidemiology: 1) severe involvement generally seen in African Americans 2) puberty to menopause in women 3) anogenital involvement more frequent in males 4) axillary involvement more frequent in females 5) prevalence 0.4% [20] Pathology: 1) keratin occlusion of apocrine duct & hair follicle 2) dilatation of apocrine duct & hair follicle rupture 3) inflammation of a single apocrine gland 4) primary abscesses are sterile, but secondary infection may occur a) Staphylococcus aureus b) Streptococci c) Escherichia coli d) Proteus mirabilis e) Pseudomonas aeruginosa 5) rupture & destruction of apocrine/eccrine/pilosebaceous unit 6) ulceration, fibrosis & sinus tract formation Genetics: - haploinsufficiency in PSENEN, PSEN1, & NCSTN (components of APP gamma-secretase) associated with [3] - SOX9 & KLF5 may be associated with hidradenitis suppurativa via their roles in epidermal differentiation & follicular inflammation [23] Clinical manifestations: 1) often begins at puberty with tender subcutaneous nodules, small papules, pustules, comedones 2) abscesses, cysts, sinus tracts (pilonidal sinuses) & scarring with more severe diseaase 3) distribution in intertriginous areas - axillary, inguinal [15], anogenital &/or inframammary 4) intermittent pain, marked point tenderness 5) erythematous surrounding 6) rarely edema of associated limb 7) severe nodulocystic acne may be present 8) scalp folliculitis may be present [2] 9) arthralgias/arthritis a) peripheral joints b) sacroiliac joint c) spine * images [9,10,15] Laboratory: - cultures are generally negative Compications: - increased risk of cancer [17] - overall cancer (RR=1.28) - Hodgkin lymphoma (RR=5.08) - oropharyngeal cancer (RR=3.10) - CNS cancer (RR=2.40) - nonmelanoma skin cancer (RR=2.06) - prostate cancer (RR=2.05) - colorectal cancer (RR=1.45) Differential diagnosis: 1) furuncle 2) carbuncle 3) lymphadenitis 4) ruptured inclusion cyst 5) lymphogranuloma venereum 6) donovanosis 7) scrofuloderma 8) actinomycosis 9) sinus tracts & fistulas associated with inflammatory bowel disease Management: 1) reduction of modifiable risk factors a) smoking cessation [2] b) weight reduction unlikely to reduce severity [2] 2) antiseptic washes (dilute bleach, chlorhexidine), warm compresses, & topical antibiotics (clindamycin) for mild disease [2] 3) antiperspirant agents (6.25% aluminum chloride hexahydrate) 4) oral antibiotics until lesions resolve (may take weeks) a) erythromycin 250-500 mg QID b) tetracycline 250-500 mg QID c) minocycline 100 mg BID d) clindamycin + rifampin 5) prednisone 70 mg PO tapered over 14 days 6) intralesional triamcinolone 0.3-0.5% diluted with lidocaine 7) oral retinoid: isotretinoin or acitretin - risk for idiopathic intracranial hypertension coadministered with doxycycline [22] 8) TNF-alpha inhibitor [2] - 40 mg adalimumab SC weekly or every other week [4,6] - no need to interrupt adalimumab treatment prior to surgery [19] 9) IL-17 inhibitors secukinumab (Cosentyx), bimekizumab (investigational) [21] 10) oral contraceptives, spironolactone (women), finasteride [2] 12) surgery is the mainstay of management [19] a) incision & drainage b) complete excision of axillary or anogenital region down to fascia

General

sweat gland disease; apocrine gland disease abscess

References

  1. Color Atlas & Synopsis of Clinical Dermatology, Common & Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY, 1997, pg 8-11
  2. Medical Knowledge Self Assessment Program (MKSAP) 11, 15, 16, 17, 18. American College of Physicians, Philadelphia 1998, 2009, 2012, 2015, 2018.
  3. Wang B et al. gamma-secretase gene mutations in familial acne inversa. Science 2010 Nov 19; 330:1065. PMID: 20929727
  4. Kimball AB et al. Adalimumab for the treatment of moderate to severe hidradenitis suppurativa: A parallel randomized trial. Ann Intern Med 2012 Dec 18; 157:846 PMID: 23247938
  5. Alikhan A, Lynch PJ, Eisen DB. Hidradenitis suppurativa: a comprehensive review. J Am Acad Dermatol. 2009 Apr;60(4):539-61 PMID: 19293006
  6. Haslund P, Lee RA, Jemec GB. Treatment of hidradenitis suppurativa with tumour necrosis factor-alpha inhibitors. Acta Derm Venereol. 2009 Nov;89(6):595-600 PMID: 19997689
  7. Al-Ali FM, Ratnamala U, Mehta TY et al Hidradenitis suppurativa (or Acne inversa) with autosomal dominant inheritance is not linked to chromosome 1p21.1-1q25.3 region. Exp Dermatol. 2010 Sep;19(9):851-3 PMID: 20698881
  8. Jemec GB. Clinical practice. Hidradenitis suppurativa. N Engl J Med. 2012;366(2):158-164. PMID: 22236226
  9. Hidradenitis suppurativa (image) American Academy of Dermatology https://www.aad.org/public/diseases/painful-skin-joints/hidradenitis-suppurativa - Mayo Clinic (image) Hidradenitis suppurativa http://www.mayoclinic.org/diseases-conditions/hidradenitis-suppurativa/multimedia/hidradenitis-suppurativa/img-20008058
  10. Jovanovic M, James WD (images) Medscape: Hidradenitis Suppurativa http://emedicine.medscape.com/article/1073117-overview
  11. Rambhatla PV, Lim HW, Hamzavi I. A systematic review of treatments for hidradenitis suppurativa. Arch Dermatol. 2012 Apr;148(4):439-46 PMID: 22184715
  12. Ingram JR et al. Interventions for hidradenitis suppurativa: A Cochrane systematic review incorporating GRADE assessment of evidence quality. Br J Dermatol 2016 May; 174:970. PMID: 26801356 - Kimball AB et al. Two phase 3 trials of adalimumab for hidradenitis suppurativa. N Engl J Med 2016 Aug 4; 375:422 PMID: 27518661 - Kohorst JJ, Baum CL, Otley CC et al Surgical Management of Hidradenitis Suppurativa: Outcomes of 590 Consecutive Patients. Dermatol Surg. 2016 Jun 21. [Epub ahead of print] PMID: 27340739
  13. Ingram JR. Hidradenitis suppurativa: an update. Clin Med (Lond). 2016 Feb;16(1):70-3. PMID: 26833521
  14. Lindhardt Saunte DM, MD, Ernst Jemec JBE Hidradenitis Suppurativa. Advances in Diagnosis and Treatment. JAMA. 2017;318(20):2019-2032 PMID: 29183082 https://jamanetwork.com/journals/jama/article-abstract/2664466
  15. Beltrame A, Staffolan S Clinical Diagnosis of Hidradenitis Suppurativa. N Engl J Med 2017; 377:2474. Dec 21, 2017 PMID: 29262280 http://www.nejm.org/doi/full/10.1056/NEJMicm1707912
  16. Micheletti RG. An update on the diagnosis and treatment of hidradenitis suppurativa. Cutis. 2015 Dec;96(6 Suppl):7-12. PMID: 27051885
  17. Jung JM, Lee KH, Kim YJ et al Assessment of Overall and Specific Cancer Risks in Patients With Hidradenitis Suppurativa. JAMA Dermatol. 2020;156(8):844-853 PMID: 32459291 PMCID: PMC7254443 Free PMC article https://jamanetwork.com/journals/jamadermatology/fullarticle/2766018
  18. Satter EK A 40-Year-Old Man With Painful, Rupturing Lesions. Medscape. July 17, 2021 https://reference.medscape.com/viewarticle/860310_3
  19. Bechara FG, Podda M, Prens EP et al Efficacy and Safety of Adalimumab in Conjunction With Surgery in Moderate to Severe Hidradenitis Suppurativa. The SHARPS Randomized Clinical Trial. JAMA Surg 2021 Nov; 156:1001 PMID: 34406349 https://jamanetwork.com/journals/jamasurgery/fullarticle/2783257
  20. Jfri A, Nassim D, O'Brien E et al Prevalence of Hidradenitis Suppurativa. A Systematic Review and Meta-regression Analysis. JAMA Dermatol. 2021;157(8):924-931. PMID: 34037678 PMCID: PMC8156162 (available on 2022-05-26) https://jamanetwork.com/journals/jamadermatology/fullarticle/2780049
  21. Bosworth T Studies Validate IL-17 as Hidradenitis Suppurativa Drug Target. Medscape. March 20, 2023 https://www.medscape.com/viewarticle/989861 - Kimball AB, Jemec GBE, Alavi A et al Secukinumab in moderate-to-severe hidradenitis suppurativa (SUNSHINE and SUNRISE): week 16 and week 52 results of two identical, multicentre, randomised, placebo- controlled, double-blind phase 3 trials. Lancet. 2023 Mar 4;401(10378):747-761. PMID: 36746171 https://www.thelancet.com/journals/lancet/article/PIIS0140-6736(23)00022-3/fulltext
  22. Goldburg SR, Strober BE, Payette MJ. Hidradenitis suppurativa: current and emerging treatments. J Am Acad Dermatol. 2020;82:1061-82. PMID: 31604100
  23. Sun Q, Broadaway KA, Edmiston SN et al Genetic Variants Associated With Hidradenitis Suppurativa. JAMA Dermatol. 2023;159(9):930-938 PMID: 37494057 PMCID: PMC10372759 (available on 2024-07-26) https://jamanetwork.com/journals/jamadermatology/fullarticle/2807701