> Table of Contents > Hidradenitis Suppurativa
Hidradenitis Suppurativa
Travis C. Geraci, MD
Siva Vithananthan, MD, FACS
image BASICS
DESCRIPTION
  • Chronic follicular occlusive disease manifested as recurrent inflammatory nodules, abscesses, sinus tracts, and complex scar formation
  • Lesions are tender, malodorous, often with exudative drainage.
  • Common in intertriginous skin regions: axillae, groin, perianal, perineal, inframammary skin
  • System affected: skin
  • Synonym(s): acne inversa; Verneuil disease; apocrinitis; hidradenitis axillaris
Geriatric Considerations
Rare after menopause
Pediatric Considerations
Rarely occurs before puberty; occurrence in children is associated with premature adrenarche.
Pregnancy Considerations
No Accutane (isotretinoin) or tetracycline treatment during pregnancy. Disease may ease during pregnancy and rebound after parturition.
EPIDEMIOLOGY
Predominant sex: female > male (3:1)
Incidence
Peak onset during 2nd and 3rd decades of life
Prevalence
0.3-4%
ETIOLOGY AND PATHOPHYSIOLOGY
  • Not fully understood; previously considered a disorder of apocrine glands
  • Inflammatory disorder of the hair follicle triggered by follicular plugging within apocrine gland-bearing skin.
  • Hormonally induced ductal keratinocyte proliferation leads to a failure of follicular epithelial shedding, causing follicular occlusion.
  • Mechanical stress on skin (intertriginous regions) precipitates follicular rupture and immune response.
  • Bacterial involvement is a secondary event.
  • Rupture and reepithelialization cause sinus tracts to form.
  • Obesity and smoking are major risk factors in disease onset and severity.
Genetics
  • Familiar occurrences suggest single gene transmission (autosomal dominant), but the condition may also be polygenic.
  • Estimated 40% of patients have an affected family member.
RISK FACTORS
  • Obesity
  • Smoking
  • Hyperandrogenism, oral contraceptive pills (OCPs)
  • Lithium may trigger onset of or exacerbate this condition.
GENERAL PREVENTION
  • Lose weight if overweight or obese.
  • Smoking cessation
  • Avoid constrictive clothing/synthetic fabrics, frictional trauma, heat exposure, excessive sweating, shaving, depilation, and deodorants.
  • Use of antiseptic soaps.
COMMONLY ASSOCIATED CONDITIONS
  • Acne vulgaris, acne conglobate
  • Perifolliculitis capitis abscedens et suffodiens (dissecting cellulitis of scalp)
  • Pilonidal disease
  • Arthritis and spondyloarthritis (seronegative)
  • Obesity (with diabetes, atopy, acanthosis)
  • Irritable bowel disease (Crohn disease)
  • Squamous cell carcinoma
  • PAPASH syndrome (pyogenic arthritis, pyoderma gangrenosum, acne, suppurative hydradenitis)
image DIAGNOSIS
PHYSICAL EXAM
  • Tender nodules (dome-shaped) 0.5 to 3 cm in size are present.
    • Location corresponds with the distribution of apocrine-related mammary tissue and terminal hair follicles dependent on low androgen concentrations.
    • Sites ordered by frequency of occurrence: axillary, inguinal, perianal and perineal, mammary and inframammary, buttock, pubic region, chest, scalp, retroauricular, eyelid
    • Large lesions are often fluctuant; comedones may be present.
    • Possible malodorous discharge
  • Hurley clinical staging system
    • Stage I: abscess formation (singular or multiple) without sinus tracts or scarring
    • Stage II: widely separated, recurrent abscesses with tract formation and scarring
    • Stage III: diffuse, multiple interconnected tracts and abscesses
  • Sartorius clinical staging system (points attributed)
    • Anatomic region
    • Quantity and quality of lesions
    • Distance between lesions
    • Presence or absence of normal skin between lesions
DIFFERENTIAL DIAGNOSIS
  • Acne vulgaris, conglobate
  • Furunculosis/carbuncles
  • Infected Bartholin or sebaceous cysts
  • Lymphadenopathy/lymphadenitis
  • Cutaneous Langerhans cell histiocytosis
  • Actinomycosis
  • Granuloma inguinale
  • Lymphogranuloma venereum
  • Apocrine nevus
  • Crohn disease with anogenital fistula(s) (may coexist with hidradenitis suppurativa)
  • Cutaneous tuberculosis
  • Fox-Fordyce disease
DIAGNOSTIC TESTS & INTERPRETATION
Initial Tests (lab, imaging)
  • Cultures of skin or aspirates of boils are most commonly negative. When positive, cultures are often polymicrobial and commonly grow Staphylococcus aureus and Staphylococcus epidermidis.
  • Lesion biopsy usually unnecessary. Useful to rule out other disorders such as squamous cell carcinoma.
  • May note increased erythrocyte sedimentation rate (ESR), leukocytosis, decreased serum iron, normocytic anemia, or changes in serum electrophoresis pattern.
Follow-Up Tests & Special Considerations
  • Consider biopsy of concerning lesions due to increased risk of squamous cell carcinoma.
  • If the patient is female, overweight, and/or hirsute, consider evaluating the following:
    • Dehydroepiandrosterone sulfate
    • Testosterone: total and free
    • Sex hormone-binding globulin
    • Progesterone
Diagnostic Procedures/Other
  • Incision and drainage, culture and biopsy
  • Ultrasound may be useful in planning an excision to identify the full extent of sinus tracts.
Test Interpretation
  • Dermis shows granulomatous inflammation and inflammatory cells, giant cells, sinus tracts, subcutaneous abscesses, and extensive fibrosis.
  • Hair follicular dilatation and occlusion by keratinized stratified squamous epithelium
image TREATMENT
Despite the prevalence of this condition, few largescale randomized controlled trials have explored the safety and efficacy of treatment. Evidence is generally of poor quality (2)[A]. Treatment goals: Reduce extent of disease, prevent new lesions, remove chronic disease, and limit scar formation.
  • Conservative treatment includes all items under general prevention, plus use of warm compresses, sitz baths, topical antiseptics for inflamed lesions, and nonopioid analgesics.
  • Weight loss and smoking cessation result in marked improvement.
  • For stage I-II, attempt medical treatment.
  • Short medical trial may be appropriate in stage III prior to moving on to surgical therapies.
  • No medications are curative; relapse is almost inevitable, but the disease may be controlled.
GENERAL MEASURES
  • Education and psychosocial support
  • Appropriate hygiene including avoidance of shearing stress to skin (light clothing), daily cleansing with antibacterial soap
  • Diet: Avoid dairy and high glycemic loads.
  • Symptomatic treatment for acute lesions
  • Improve environmental factors that cause follicular blockage (see “General Prevention”).
  • Smoking cessation and weight loss
P.485

MEDICATION
First Line
  • Stage I disease: Consider either systemic or topical antibiotics.
    • Topical antibiotics (clindamycin has the most evidence) (3)[B]
      • Clindamycin 1% solution BID for 12 weeks with or without Benzoyl peroxide 5-10% solution
      • Chlorhexidine 4% solution
    • Systemic antibiotics (initial 7- to 10-day course)
      • Tetracycline 500 mg BID
      • Doxycycline 100 mg q12h
      • Augmentin 875 mg q8-12h
      • Clindamycin 300 mg BID (4)[B]
  • Stage II-III disease
    • Address overlying bacterial infection with broadspectrum coverage. Base antibiotic selection on disease location, characteristics; longer durations (3 to 6 months) may be required.
    • Tetracycline 500 mg BID
    • Minocycline 100 mg BID
    • Doxycycline 100 mg BID
    • Minor surgical procedures (punch débridement, local unroofing) to treat individual lesions or sinus tracts
  • Other modalities (rarely used)
    • Hormonal therapy: antiandrogenic therapy such as cyproterone acetate (may not be available in the United States), estrogen/norgestrel oral contraceptive, finasteride (5 mg daily) (5)[B]
  • Intralesional corticosteroids: accelerates healing, although efficacy not formally evaluated (triamcinolone acetonide 5 to 10 mg/mL)
Second Line
  • Combination of antibiotic regimens
    • Clindamycin and rifampin
    • Rifampin, moxifloxacin, and metronidazole
  • Dapsone 50 to 150 mg daily (6)[C]
  • Metformin: significant reduction in Sartorius score (7)[B]
  • Oral retinoids (Isotretinoin): poor efficacy, limited therapeutic effect (8)[B]
  • TNF-&agr; inhibitors:
    • Infliximab: a majority of patients in the treatment group had a 50% or greater decrease in disease (9)[B]
    • Etanercept: no difference versus placebo (10)[A]
    • Adalimumab: limited efficacy
ISSUES FOR REFERRAL
  • Lack of response to treatment, stage II-III disease, or concern for malignancy (squamous cell carcinoma) is a reason to refer for surgical excision or radiation/laser treatment (stage II).
  • If significant psychosocial stress exists secondary to disease, refer for stress management or psychiatric evaluation.
  • Suspicion of hyperandrogenic states (e.g., polycystic ovary syndrome [PCOS]) should prompt investigation or referral.
  • Severe perianal/perivulvar disease or otherwise very extensive disease may prompt referral to plastic surgeon or reconstructive urologist.
SURGERY/OTHER PROCEDURES
  • Important mode of treatment
  • Could be used in conjunction with antibiotics or if first-line therapy fails
  • Various surgical approaches have been used for stage II-III disease (11,12)[B]:
    • Incision and drainage: may be necessary to treat as a temporizing method for acute flare-ups
    • Deroofing and marsupialization of the sinus tracts is often beneficial primarily for Hurley stage I-II disease, as healing time is reduced. Recurrences remain common but usually are smaller than original lesions (13)[B].
    • 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 to 72 months): axillary (3%), perianal (0%), inguinoperineal (37%), submammary (50%)
  • Laser therapy for Hurley stage I-II disease (rarely used)
    • Consider monthly treatments with neodymiumdoped yttrium aluminum garnet (Nd:YAG) laser for 3 to 4 months.
    • CO2 laser ablation with healing by secondary intention
  • Cryotherapy and photodynamic therapy have shown variable results, they are not routinely recommended.
image ONGOING CARE
FOLLOW-UP RECOMMENDATIONS
Follow up monthly or sooner to evaluate progress and to assist with symptom management.
DIET
  • Avoid dairy, high glycemic loads.
  • Healthy diet that promotes weight loss.
  • May benefit from zinc supplementation
PATIENT EDUCATION
  • Severity can range from only 2 to 3 papules per year to extensive draining sinus tracts.
  • Medications are temporizing measures, rarely curative. Attempts at local surgical “cures” do not affect recurrence at other sites.
  • Smoking cessation and weight loss can improve symptoms significantly. Hidradenitis Suppurativa Foundation: www.hs-foundation.org
PROGNOSIS
  • Individual lesions heal slowly in 10 to 30 days.
  • Recurrences may last for several years.
  • Relentlessly progressive scarring and sinus tracts are likely with severe disease.
  • Radical wide-area excision, with removal of all hair-bearing skin in the affected area, shows the greatest chance for cure.
REFERENCES
1. Hidradenitis Suppurativa Foundation. http://www.hs-foundation.org.
2. Rambhatla PV, Lim HW, Hamzavi I. A systematic review of treatments for hidradenitis suppurativa. Arch Dermatol. 2012;148(4):439-446.
3. Jemec GB, Wendelboe P. Topical clindamycin versus systemic tetracycline in the treatment of hidradenitis suppurativa. J Am Acad Dermatol. 1998;39(6):971-974.
4. van der Zee HH, Boer J, Prens EP, et al. The effect of combined treatment with oral clindamycin and oral rifampicin in patients with hidradenitis suppurativa. Dermatology. 2009;219(2):143-147.
5. Searles GE. Daily oral finasteride 5 mg for hidradenitis suppurativa. Paper presented at: Annual Meeting of the Canadian Dermatology Association; July 3, 2009; Vancouver, British Columbia, Canada.
6. Kaur MR, Lewis HM. Hidradenitis suppurativa treated with dapsone: a case series of five patients. J Dermatolog Treat. 2006;17(4):211-213.
7. Verdolini R, Clayton N, Smith A, et al. Metformin for the treatment of hidradenitis suppurativa: a little help along the way. J Eur Acad Dermatol Venereol. 2013;27(9):1101-1108.
8. Soria A, Canoui-Poitrine F, Wolkenstein P, et al. Absence of efficacy of oral isotretinoin in hidradenitis suppurativa: a retrospective study based on patients' outcome assessment. Dermatology. 2009;218(2):134-135.
9. Grant A, Gonzalez T, Montgomery MO, et al. Infliximab therapy for patients with moderate to severe hidradenitis suppurativa: a randomized, double-blind, placebo-controlled crossover trial. J Am Acad Dermatol. 2010;62(2):205-217.
10. Adams DR, Yankura JA, Fogelberg AC, et al. Treatment of hidradenitis suppurativa with etanercept injection. Arch Dermatol. 2010;146(5):501-504.
11. Blok JL, van Hattem S, Jonkman MF, et al. Systemic therapy with immunosuppressive agents and retinoids in hidradenitis suppurativa: a systematic review. Br J Dermatol. 2013;168(2):243-252.
12. Kagan RJ, Yakuboff KP, Warner P, et al. Surgical treatment of hidradenitis suppurativa: a 10-year experience. Surgery. 2005;138(4):734-740; discussion 740-741.
13. van der Zee HH, Prens EP, Boer J. Deroofing: a tissue-saving surgical technique for the treatment of mild to moderate hidradenitis suppurativa lesions. J Am Acad Dermatol. 2010;63(3):475-480.
Additional Reading
&NA;
  • Alikhan A, Lynch PJ, Eisen DB. Hidradenitis suppurativa: a comprehensive review. J Am Acad Dermatol. 2009;60(4):539-561.
  • Jemec GB. Clinical practice. Hidradenitis suppurativa. N Engl J Med. 2012;366(2):158-164.
  • Mandal A, Watson J. Experience with different treatment modules in hidradenitis suppuritiva: a study of 106 cases. Surgeon. 2005;3(1):23-26.
  • Slade DE, Powell BW, Mortimer PS. Hidradenitis suppurativa: pathogenesis and management. Br J Plast Surg. 2003;56(5):451-461.
Codes
&NA;
ICD10
L73.2 Hidradenitis suppurativa
Clinical Pearls
&NA;
  • Chronic inflammatory disease of the skin, often difficult to control with behavior changes and medication alone
  • First-line treatment for mild disease is topical and/or systemic antibiotics.
  • For patients with refractory or severe disease, wide local excision provides the only chance at a cure. Success rates depend on the location and extent of excision.