Hidradenitis Suppurativa– Causes, Symptoms, Diagnosis, Treatment and Ongoing care

Basics

Description

  • Acute, tender, cystlike abscesses in apocrine gland–bearing skin (axillae, anogenital area, pubes, areolae; also apocrine glands scattered around umbilicus, scalp, trunk, and face); often a chronic condition
  • Over time, fibrotic sinus tracts develop with intermittent drainage and periodic acute abscesses.
  • Common from late puberty through 40 years
  • System(s) affected: Skin/Exocrine
  • Synonym(s): Acne inversa; Apocrinitis; Hidradenitis axillaris

Geriatric Considerations

Rare after menopause

Pediatric Considerations

Rarely occurs before puberty

Pregnancy Considerations

No isotretinoin (Accutane) or tetracycline treatment during pregnancy. Disease may ease during pregnancy and rebound following parturition.

Epidemiology

  • Predominant age: Peak onset age 11–30 years, commonly 30–40 years
  • Predominant sex: 3:1 female-to-male ratio

Incidence

Peaks during 2nd and 3rd decades of life

Prevalence

0.3–4%

Risk Factors

  • Obesity
  • Acne
  • Hyperandrogenism
  • Hirsutism
  • Smoking
  • Lithium may trigger onset or exacerbate this condition.

Genetics

Unknown; possibly single gene transmission (autosomal dominant), possibly polygenic

General Prevention

  • Weight loss if overweight or obese
  • Avoid constrictive clothing/frictional trauma.
  • Avoid heat exposure, sweating, shaving, depilation, and deodorants.
  • Use antiseptic soaps, topical application of tea tree oil
  • Smoking cessation

Etiology

  • Traditionally considered a disorder of apocrine glands, but now believed to be primarily an inflammatory disorder of the hair follicle triggered by follicular plugging within apocrine gland–bearing skin
  • Bacterial involvement is not a primary pathogenic event, but secondary.
  • Sebum excretion not an important factor.
  • Smoking may be a major triggering factor.
  • Considered part of follicular occlusive tetrad: Acne conglobata, dissecting cellulitis of scalp, hidradenitis suppurativa, and pilonidal sinus
  • There may be a genetic predisposition component that is still being elucidated.

Commonly Associated Conditions

  • Acne
  • Perifolliculitis capitis abscedens et suffodiens (dissecting cellulitis of scalp)
  • Arthritis
  • Obesity with associated diabetes mellitus, atopy, acanthosis nigricans
  • Crohn disease
  • Pilonidal disease
  • Smoking

Accutane, Sebaceous gland, Skin Disorder, apocrine glands, inflammatory disorder, depilation, chronic condition, deodorants, heat exposure,

Diagnosis

This is a clinical diagnosis.

History

  • Multiple recurrences at the same site
  • Recurrent deep boils >6 months in flexural sites
  • Onset after puberty
  • Poor response to conventional antibiotics
  • Strong tendency toward relapse or recurrence
  • Improved with smoking cessation and weight loss
  • Personal or family history of acne or pilonidal sinuses and premenstrual exacerbation of boils
  • Early signs are pruritus, erythema, and local hyperhidrosis.
  • Healing sites may be accompanied by scarring and sinus tracts.
  • Associated arthritis and arthropathy, especially the knees

Physical Exam

  • Tender nodules (dome-shaped) 0.5–3 cm in size are present:
    • Distribution is over the area of apocrine glands, with axillae and groin being most common. Sites ordered by frequency of occurrence: Axillary, inguinal, perianal and perineal, mammary and inframammary, buttock, pubic region, chest, scalp, retroauricular, eyelid.
    • Large lesions often are fluctuant.
    • Comedones may be present.
    • Possible malodorous discharge
  • Staging: Most commonly done with the “Hurley” staging system, although it is likely that future studies will use the more complicated “Sartorius” staging system for clinical research:
    • Hurley staging system:
      • Stage I: Abscess formation (singular or multiple) without sinus tracts or scarring
      • Stage II: One or more widely separated recurrent abscesses with tract formation and scars
      • Stage III: Multiple interconnected tracts and abscesses throughout an entire area

Diagnostic Tests & Interpretation

Lab

Initial lab tests

  • Cultures of skin or aspirates of boils often show no pathogens.
  • When positive, deep cultures are often polymicrobial and have shown Staphylococci aureus, S. epidermidis, Streptococcus milleriS. hominisBacteroides fragilis, and B. melaninogenicus.
  • Check sensitivities due to increasing antibiotic resistance.
  • May note increased erythrocyte sedimentation rate (ESR), leukocytosis, decreased serum iron, normocytic anemia, and changes in serum electrophoresis pattern, probably due to chronic inflammatory process
  • Consider these additional studies at baseline, depending on elected treatment:
    • Complete blood cell count with differential
    • Basic metabolic panel plus magnesium
    • Liver function tests
    • Glucose-6-phosphate dehydrogenase level
    • Fasting lipids
  • Purified protein derivative

Follow-Up & Special Considerations

If patient is female with hirsutism, check:

  • Dehydroepiandrosterone sulfate
  • Testosterone total and free
  • Sex-hormone-binding globulin
  • Progesterone

Diagnostic Procedures/Surgery

  • Incision and drainage of lesion(s) with culture and biopsy
  • Mortimer clinical criteria for diagnosis: Recurrent boils in apocrine-gland bearing skin for more than 3 months, presence of comedones in apocrine skin or retroauricular sites, and exacerbation of disease premenstrually (1)
  • Ultrasound may be useful to assist in planning an entire excision to identify the full extent of sinus tracts.

Pathological Findings

  • Histologically, chronic disease shows a dermis that contains inflammatory cells, giant cells, sinus tracts, subcutaneous abscesses, and later, extensive fibrosis.
  • Multiple comedones and follicular dilatation and possible occlusion by keratinized stratified squamous epithelium may be observed.

Differential Diagnosis

  • Furunculosis/carbuncles: Differentiate by specific culture and also by the response to specific antibiotics.
  • Infected Bartholin or sebaceous cysts
  • Hyperandrogenism
  • Lymphadenopathy/lymphadenitis
  • Cutaneous Langerhans cell histiocytosis
  • Actinomycosis
  • Granuloma inguinale
  • Lymphogranuloma venereum
  • Apocrine nevus
  • Crohn with anogenital fistula (may coexist with hidradenitis suppurativa or be mistaken for it)
  • Cutaneous tuberculosis
  • Fox-Fordyce disease

Treatment

  • Conservative treatment includes all items under general prevention, plus use of warm compresses, sitz baths, and topical antiseptics for inflamed lesions and non-narcotic analgesics (2)[C]. Additionally, weight loss and smoking cessation have shown improvement (3).
  • Medical treatment is often tried first because of extensive nature of surgery treatment.
  • No medications are curative. Relapse almost inevitable once medication is stopped. Strategy is to keep disease contained with medications. If a cure is sought and disease is Hurley stage II–III, surgery should be considered.
  • Because of the significant psychosocial stressful nature of this illness, referral of patient for stress management and to a support group is recommended.

Medication

First Line

  • Stage I disease; consider either systemic or topical antibiotics:
    • Topical antibiotics: Select one of the following (clindamycin has the most evidence):
      • Clindamycin 1% solution b.i.d. (× 12 wks at a minimum) (2,4)
      • Benzoyl peroxide 5–10% soln b.i.d. (4)[C]
      • Chlorhexidine 4% soln b.i.d. (4)[C]
    • Systemic antibiotics: Select one of the following:
      • Tetracycline 500 mg b.i.d. (>12 weeks) (5)
      • Oral clindamycin 300 mg b.i.d. and rifampin 300 mg b.i.d. for 12 wks (2,6)[B]
  • Stage II–III disease:
    • Antibiotic therapy is usually systemic and mostly aimed at addressing likely overlying bacterial infection, with a need to cover gram-negative, gram-positive, and anaerobic bacterial infections, with the antibiotic selection based upon lesion location and characteristics.
    • While antibiotic therapy is generally attempted, it is more likely that more aggressive approaches will be needed subsequently. While this usually implies surgical treatment, medical management described below may lead to progress once any infectious component has been addressed:
      • Immunosuppressive therapies with infliximab (2,7)[C]

Second Line

  • Stage I:
    • Consider oral contraceptives for women if antibiotics fail. Low-dose progesterone birth control pills (e.g., Norinyl, Ortho-Novum) (2)[C].
    • Sulfamethoxazole/trimethoprim DS b.i.d. (4).
    • Oral amoxicillin 250–500 mg b.i.d., tetracycline, minocycline 50–100 mg b.i.d., doxycycline 50–100 mg b.i.d., or erythromycin (2)[C]
    • Limited lesions can be injected with corticosteroids, and flares can be addressed with short courses of oral or intralesional corticosteroids such as triamcinolone (2)[C].
    • Dapsone (2)[C]
    • Finasteride (consider only postmenopausally for women) (2,4)[C]
  • Stage II–III disease or recalcitrant Stage I:
    • Systemic retinoids: Isotretinoin 40–80 mg p.o. every day for 4 months (2)[C]
    • Cyproterone acetate (if available) (2)[B]

Additional Treatment

General Measures

  • Symptomatic treatment for acute lesions
  • Prevention of new lesions
  • Local cleansing (germicidal soap)
  • Improve environmental factors that cause follicular blockage (see General Prevention).
  • Steps that may improve the disease are smoking cessation and weight loss if overweight or obese.

Issues for Referral

Lack of response to treatment or stage II–III disease is a reason to refer for surgical excision or radiation/laser treatment (stage II).

Surgery/Other Procedures

  • Various surgical approaches have been used for stage II–III disease:
    • Incision and drainage is discouraged as it is rarely curative (2,8)[C].
    • Deroofing and marsupialization of the sinus tracts may be of benefit, as healing time is reduced but recurrences seen (2,8)[C]
    • Treatment for stage III or intractable cases: Wide full-thickness excision with healing by granulation or flap placement is the most definitive treatment and rarely has local recurrence if all sinus tracts are excised. Rates of local recurrence (within 3–72 months) are: Axillary (3%) and perianal (0%), inguinoperineal (37%), and submammary (50%) (9,10).
  • CO2 laser ablation with healing by secondary intention has recent data with good results (11).

Ongoing Care

Follow-Up Recommendations

Monthly or sooner follow-up to evaluate progress and assist with symptom management

Patient Monitoring

Regular follow-up visits

Diet

No limitations

Patient Education

  • Severity can range from only 2 or 3 papules per year to extensive draining sinus tracts.
  • Medications are temporizing measures and are rarely curative. Smoking cessation and weight loss can improve symptoms significantly.
  • Attempts at local surgical “cures” do not affect recurrence at other sites.
  • Hidradenitis suppurativa is considered a misnomer by many. Acne inversa is the more descriptive terminology.
  • Hidradenitis Suppurativa Foundation: http://www.hs-foundation.org

Prognosis

  • Individual lesions (with or without drainage) heal slowly in 10–30 days.
  • Recurrences may last for several years.
  • Rare spontaneous resolution
  • Relentlessly progressive scarring and sinus tracts are likely for chronic severe disease.
  • Radical wide-area excision is the method that has shown the greatest likelihood for a lack of recurrence up to 5 years.

Complications

  • Contracture formation at the sites of excisions in the case of surgery with possibly restricted limb mobility
  • Rarely, squamous cell carcinoma may develop in indolent sinus tracts (usually anogenital).
  • Disseminated infection septicemia (unusual)
  • Lymphedema
  • Urethral/rectal fistula
  • Anemia
  • Arthritis:
    • Asymmetrical pauciarticular to symmetrical polyarthritis/polyarthralgia in larger joints
  • Amyloidosis and hypoproteinemia can lead to renal failure and even death.
  • Interstitial keratitis
  • Lumbosacral epidural abscess
  • Sacral bacterial osteomyelitis

References

1. Mortimer PS, Dawber RP, Gales MA, et al. Mediation of hidradenitis suppurativa by androgens. Br Med J (Clin Res Ed). 1986;292:245–8.

2. Lam J, Krakowski AC, Friedlander SF. Hidradenitis suppurativa (acne inversa): management of a recalcitrant disease. Pediatr Dermatol. 2007;24:465–73.

3. Sartorius K, Emtestam L, Jemec GB, Lapins J. Objective scoring of hidradenitis suppurativa reflecting the role of tobacco smoking and obesity. The British Journal of Dermatology, 2009;161(4):831–9.

4. Lee RA, Yoon A, Kist J. Hidradenitis suppurativa: an update. Adv Dermatol. 2007;23:289–306.

5. Jemec GB, Wendelboe P. Topical clindamycin versus systemic tetracycline in the treatment of hidradenitis suppurativa. J Am Acad Dermatol. 1998;39:971–4.

6. Mendonça CO, Griffiths CE. Clindamycin and rifampicin combination therapy for hidradenitis suppurativa. Br J Dermatol. 2006;154:977–8.

7. Grant A, Gonzalez T, Montgomery MO, Cardenas V, Kerdel FA. Infliximab therapy for patients with moderate to severe hidradenitis suppurativa: a randomized, double-blind, placebo-controlled crossover trial. J Am Acad Dermatol. 2010;62:205–217.

8. Slade DE, Powell BW, Mortimer PS. Hidradenitis suppurativa: pathogenesis and management. Br J Plast Surg. 2003;56:451–61.

9. Kagan RJ, Yakuboff KP, Warner P, et al. Surgical treatment of hidradenitis suppurativa: a 10-year experience. Surgery. 2005;138:734–40; discussion 740–1.

10. Mandal A, Watson J. Experience with different treatment modules in hidradenitis suppuritiva: a study of 106 cases. Surgeon. 2005;3:23–6.

11. Hazen PG, Hazen BP. Hidradenitis Suppurativa: Successful Treatment Using Carbon Dioxide Laser Excision and Marsupialization. Dermatol Surg. 2010;36:208–213.

Codes

ICD9

705.83 Hidradenitis

Snomed

59393003 Hidradenitis suppurativa (disorder)

Clinical Pearls

  • Hidradenitis suppurativa is an inflammatory disease of the apocrine skin areas that can be difficult to contain with behavior changes and medication.
  • For those people with recalcitrant or severe disease, surgery provides the only treatment with a chance at a cure. Succe

Jean-Paul Marat

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