Suppurative hidradenitis L73.2

Authors: Prof. Dr. med. Peter Altmeyer, Duygu Percin

All authors of this article

Last updated on: 02.02.2021

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Synonym(s)

Abscès tubereux de l'aiselle; acne inversa; Acnetetrade; Apocrine acne; apocrine axillary abscess; Axilla abscess; Axillary abscess apocrine; Chronic recurrent hidradenitis; Dissecting terminal hair folliculitis; follicular occlusion syndrome; Follicular occlusion triad; Hidrosadenitis; Intertriginous acne; pyodermia fistulans sinifica; sweat gland abscess; Sweat gland abscess; Sweat gland abscess apocrine; Verneuil`s disease; Verneuil's disease

History
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Velpeau, 1839; Verneuil, 1854; Plewig and Steger, 1989

Definition
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Chronic recurrent, melting and scarring, furunculoid inflammation of the skin and subcutis, which manifests itself preferably in the intertriginous areas, i.e. perianal, inguinal and/or axillary.

Occurrence/Epidemiology
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In Denmark, the prevalence in the general population is 1.1%. In England the incidence is 1:600. Africans have higher incidences than Europeans.

Etiopathogenesis
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Chronic folliculitis with destruction of the terminal hair follicles and secondary involvement of the apocrine sweat glands

Heredity and inheritance are controversial.

In some patients, follicular hyperkeratosis with subsequent superinfection is detected (see below acne inversa; acne triad or acne tetrade).

In other patients, however, follicular hyperkeratosis cannot be detected. Otherwise, the clinical picture is largely identical.

Associations with Crohn's disease have been described - risk tripled (Garg et al 2018) - as well as with rheumatoid factor-negative polyarthritis, hypertension and pyoderma gangränosum. (knee, ankle and elbow joints); rarely also with systemic amyloidosis.

Obviously, HS patients have an increased cardiovascular risk (myocardial infarction, ischemic stroke)

Favouring factors: smoking (90% of patients are smokers!), sweating, obesity (odds ratio 3.9), metabolic snydrome (Phan K etal. 2019), abrasive clothing, regular shaving of the armpit hairs, depilatory externals.

Manifestation
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In a larger American study (Garg A et al. 2018) the age of onset of the disease was between 28 and 64 years; 56.5%) were between 18 - 44 years old, 34.2% between 45 - 64 years, 9.3% were > 65 years old. Perianal manifestation occurs more frequently in men than in women. Also more frequent in smokers.

Localization
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Axilla, inguinal region, anal and perianal region, perineum, scrotum, buttocks, inner and extensor sides of the thigh, upper arm region. Rarely nipple area and vulva.

Clinical features
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In the early stages, some cases show inflammatory, superficial, bright red, painful nodules and nodules. These can confluent into painful, bulbous abscesses or break open in a purulent manner.

Depending on the clinical manifestation, 3 degrees of severity (Hurley) are distinguished:

  • Grade I: Isolated, single or multiple painful abscesses, no scar strands.
  • Grade II: Recurrent painful abscesses with stranding and scarring, single or multiple, but no extensive scarring.
  • Grade III: Diffuse, plate-like, inflammatory, painful infiltrations, or multiple strands and abscesses connected to each other. Danger of joint contractures due to pain-related restriction of movement.

Differential diagnosis
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Complication(s)
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Recurrences are also possible after radical surgery. The recurrence rate varies depending on the localisation: axillary approx. 3%, inguino-perineal approx. 35%, submammary approx. 50%. With longer courses, the formation of squamous cell carcinomas (Marjolin ulcer) is possible.

Furthermore, dermal contractures with movement restriction of the shoulder and hip joints, persistent swelling of the external genitals, deep pararectal fistulas in the perianal region, urethral fistulas in the genital region may remain. Septic courses are rare.

Therapy
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Grade I:

  • In early uncomplicated hidradenitis suppurativa intralesional triamcinolone crystal suspension injections 5-10 mg (e.g. with Volon A) are indicated. Abscess incision with subsequent drainage (e.g. insertion of a polyvidon-iodine-soaked ointment strip) in case of fluctuation and threatening perforation. Accompanying system antibiotic treatment with tetracyclines (Tetracycline Wolff 1.0-1.5 g/day p.o.), doxycycline (e.g. Doxycycline Stada 100-200 mg/day p.o.) for 14 days until the inflammatory symptoms subside.
  • Alternatively: Ciprofloxacin (e.g. Ciprobay 2 times/day 250 mg p.o.) or cephalosporins like cefadroxil (e.g. Cedrox 1,0-1,5 g/day). After receiving the culture result, therapy regime according to the antibiogram.
  • Alternative: The studies PIONEER I and PIONEER II confirmed a good efficiency of Adalimumab in this disease pattern (Saunte DML 2017).
  • Prophylaxis: After healing, regular treatment of the affected areas with disinfectants and deodorants, e.g. with 15-20% alcoholic aluminium chloride hexahydrate solution R005 or R006 or gel R004. Caution! Not all patients tolerate deodorants based on aluminium chloride! Important: Avoid wearing tight-fitting clothes such as T-shirts, blue jeans, body shirts, etc. Do not use deodorant rollers or deodorant sticks.

Grade II:

  • Surgical therapy: Depending on the localisation, the first choice therapy is radical surgical repair of the inflammatory affected areas (Kirschke J et al. 2015). To what extent preoperatively a therapy with isotretinoin (e.g. acne normin) 0.5-1.0 mg/kg bw p.o. over 3-6 months should be applied has not yet been decided (Blok JL et al. 2013). The German guidelines do not provide for this. Overall, the success of isotretinoin in hidradenitis suppurativa can be described as rather disappointing (Scheinfeld N 2013).
  • In case of axillary localization: Oval-lanceolate incision of the secreting glandular areas previously marked by the Minor sweat test. For this purpose aqueous iodine solution is applied to the axilla with a swab and then powdered with wheat starch; the secreting areas are marked blue-black. Excision is followed by subcutaneous mobilization of the wound edge and removal of the inflammatory conglomerates with the dissecting scissors. If technically possible, primary wound closure. Postoperative antibiotic treatment. A dressing to prevent abduction movements should immobilize the arm for about 7 days. Primary wound closure is often not possible. In these cases, closure can be attempted via mesh graft or the surgical field can be left open. Regular dressings with alginates (e.g. Algosteril, Tegagel), accompanying antibiosis. After appropriate wound granulation, mesh graft transplantation.

Grade III:

  • Surgical therapy: In cases of severe areal hidradenitis of the axillae or genitoanal region, radical excision of the inflammatory field is the method of choice. If possible start 3-6 months before with isotretinoin (e.g. isotretinoin-ratiopharm; acne normin) 0.5-1.0 mg/kg bw p.o. If necessary, intravenous antibiotic therapy (e.g. ceftriaxone once/day 2 g i.v.) should be administered over 7-10 days preoperatively. The operations should be performed in clinics that have the necessary experience in this field. Details of the surgical procedure are identical to the procedures for grade II suppurative hidradenitis. Depending on the radicality of the operation, secondary healing shows a recurrence rate of up to 30%. In principle, the more radical the operation, the lower the risk of relapse!

General therapy
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After healing, regular treatment of the affected areas with disinfectants and deodorants, e.g. with 15-20% alcoholic aluminium chloride hexahydrate solution or gel(R004). Caution! Not all patients tolerate deodorants based on aluminium chloride!

Important: Avoid wearing tight-fitting clothes such as T-shirts, tight-fitting jeans, body shirts, etc.

Do not use deodorant rollers or deodorant sticks.

Internal therapy
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  • Adalimumab: a systemic therapy with Adalimumab (Humira®: 40mg s.c./1 x per week - PiONEER I study) leads to a good clinical result after a period of 12 weeks. Adalimumab is approved for this indication (dosage as indicated above). Dosage for adults with moderate to severe acne inversa: Humira® 80 mg / 0.8 ml: induction 2 injections 80 mg (week 0: 160 mg), maintenance: from week 2 on one injection 80 mg every other week.
  • Experimental: Etanercept (2 times/week 25/50 mg s.c.) closed. Effectiveness is doubtful.
  • Experimental: The use of cyproterone acetate (Diane-35, Androcur-10) is rather disappointing in women, as is spironolactone.
  • Experimental: Therapeutic approaches with Infliximab (®, 3 infusions, 5 mg/kg bw in week 0, 2, 6; followed by a 1-year observation period) are evaluated as positive in some cases.
  • Experimental: Therapy approaches with fumaric acid ester (Deckers IE et al. 2015)
  • Experimental: focal therapy with botulinum toxn -A. Further studies are needed to determine the data available on this therapeutic approach.

Progression/forecast
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In the best case only once solitary abscess formation. Untreated chronic progressive course. The affected areas can also extend beyond the axillary and ileoinguinal region, e.g. to the upper arms, thighs and buttocks. The tendency to relapse is very high even after a period of passive healing.

Note(s)
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  • The clinical picture was first described by Velpeau in 1839. 1854 fundamental work by Verneuil; his opinion: Hidradenitis suppurativa is the result of inflamed sweat glands. Lane and Brunsting suspected a dependence on acne. In 1989 Plewig and Steger coined the term "Acne inversa", which is controversial for this clinical picture.
  • There is reason to assume that the clinical term Hidradenitis suppurativa does not conceal a clinical entity, but that different causalities lead to a largely identical clinical end result. Probably the clinical picture of acne inversa is etiopathogenetically (and also clinically: proof of comedone formation!) different from (idiopathic) Hidradenitis suppurativa. This would also justify different therapeutic approaches!
  • Smoking seems to be pathogenetically significant. In a larger study, 92% of those questioned were smokers!
  • Isotretinoin seems to have no therapeutic benefit. In the case of acitretin, positive effects seem to be detectable with long-term therapy (Zoubulis CC et al. 2015).

Literature
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  1. Aadams DR et al (2010) Treatment of hidradenitis suppurativa with etanercept injection. Arch Dermatol 146: 501-504
  2. Blok JL et al (2013) Systemic therapy with immunosuppressive agents and retinoids in hidradenitis suppurativa: a systematic review. Br J Dermatol 168:243-252.
  3. Bocchini S et al. (2003) Gluteal and perianal hidradenitis suppurativa: surgical treatment by wide excision. Dis Colon Rectum 46: 944-949
  4. Brunsting H (1939) Hidradenitis suppurativa: abscess of the apocrine sweat glands. Arch Derm Syphil 39: 108-120
  5. Cusack C et al (2006) Etanercept: effective in the management of hidradenitis suppurativa. Br J Dermatol 154: 726-729
  6. Deckers IE et al. (2015) Fumarates, a new treatment option for therapy-resistant hidradenitis suppurativa
    :a prospective open-label pilot study.Br J Dermatol 172:828-829.
  7. Egeberg A et al.(2015) Risk of Major Adverse Cardiovascular Events and All-Cause Mortality in Patients With Hidradenitis Suppurativa. JAMA Dermatol 152:429-434.
  8. Feito-Rodríguez M et al.(2009) Prepubertal hidradenitis suppurativa successfully treated with botulinum toxin A. Dermatol Surg 35:1300-1302.
  9. Garg A et al. (2018) Overall and Subgroup Prevalence of Crohn's Disease Among Patients With HidradenitisSuppurativa
    : A Population-Based Analysis in the United States. JAMA Dermatol 154:814-818. Hsiao J et al (2010) Hidradenitis suppurativa and concomitant pyoderma gangrenosum. Arch Dermatol 146: 1265-1270
  10. Jemec GB (2003) Hidradenitis suppurativa. J Cutan Med Surg 7: 47-56
  11. Katsanos KH et al (2002) Axillary hidradenitis suppurativa successfully treated with infliximab in a Crohn's disease patient. Am J Gastroenterol 97: 2155-2156
  12. Kimball AB et al. (2016) HiSCR (Hidradenitis Suppurativa Clinical Response): a novel clinical endpoint to evaluate therapeutic outcomes in patients with hidradenitis suppurativa from the placebo-controlled portion of a phase 2 adalimumab study. J Eur Acad Dermatol Venereol 30: 989-994.
  13. Khoo AB et al.(2014) Hidradenitis suppurativa treated with Clostridium botulinum toxin A. Clin Exp Dermatol 39:749-750.
  14. Kirschke J et al. (2015) Hidradenitis suppurativa/acne inversa: An update.
    Dermatologist 66: 413-422.
  15. McMillan K (2014) Hidradenitis suppurativa: number of diagnosed patients, demographic characteristics, and treatment patterns in the United States. Am J Epidemiol 179:1477-1483
  16. Mekies JR et al (2008) Long-term efficacy of a single course of infliximab in hidradenitis suppurativa. Br J Dermatol 158: 370-374
  17. Pérez Diaz D et al (1995) Squamous cell carcinoma complicating perianal hidradenitis suppurativa. Int J Colorectal 10: 225-228
  18. Phan K etal. (2019) Hidradenitis suppurativa and metabolic syndrome - systematic review and adjusted meta-analysis. Int J Dermatol in press.
  19. Plewig G, Steger M (1989): Acne inversa in: Acne and related disorders. Marks R, Plewig G (eds), Martin Dunitz Ltd, London, pp 345-347.
  20. Roy M et al (1997) Probable association between hidradenitis suppurativa and Crohn's disease:significance of epithelioid granuloma. Br J Surg 84: 375-376
  21. Saunte DML (2017) Hidradenitis suppurativa: advances in diagnosis and treatment. JAMA 318:2019-2032.

  22. Scheinfeld N (2013) Hidradenitis suppurativa: A practical review of possible medical treatments based

  23. on over 350 hidradenitis patients. Dermatol Online J 19(4):1.
  24. Sorio A et al. (2009) Absence of efficacy of oral isotretinoin in hidradenitis suppurativa: a retrospective study based on patient outcome assessment. Dermatology 218:134-135
  25. Velpeau A (1839) In: Aissele: Dictionnaire de Medicine, on Repertoire General des Sciences Medicales sons de Rapport Theorique et Pratique (Behcet Jeune Z ed) Vol. 2: 91.
  26. Verneuil A (1854): Etudes sur les tumeurs de la peau et quelques maladies de glandes sudoripares. Arch Gen Med 94: 693-705
  27. Zouboulis CC et al (2015) European S1 guideline for the treatment of hidradenitis suppurativa/acne inversa. J Eur Acad Dermatol Venereol 29:619-644.
  28. Zouboulis CC et al. (w2015) Hidradenitis Suppurativa/Acne Inversa: Criteria for Diagnosis, SeverityAssessment
    , Classification and Disease Evaluation. Dermatology 231:184-190 .

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Last updated on: 02.02.2021