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Hidradenitis Suppurativa

Chipping Away at Causes and Cures

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Vol. 18 • Issue 6 • Page 31

Hidradenitis suppurativa, often known as acne inversa, is a chronic skin condition that is difficult to manage and difficult to live with. It is seldom addressed carefully in the curricula of healthcare providers, and it is seldom discussed in the lay literature.1 Many patients feel isolated by the condition, and many healthcare providers resort to nonevidence-based methods to treat it.

The prevalence of hidradenitis suppurativa is underestimated because it is seldom a primary or presenting complaint. The condition is often an incidental finding in a routine physical, well woman or contraceptive visit. The typical clinical appearance is as an inflamed papule, nodule or abscess that is tender to the touch. It may not produce pain until pressure is applied. The usual locations are the groin, genitofemoral area, the perineum, the buttocks, the axillae and abdominal or inframammary skin folds (Figures 1 and 2).2-4 In severely affected patients, lesions may also occur in the scalp.2-4

Scope of the Problem

Several other names for hidradenitis suppurativa are used in the professional literature. The most common term is acne inversa (especially in the dermatology literature), but terms such as apocrinitis, hidradenitis axillaries, and abscess of the apocrine sweat glands have also been used.5 The latter terms are less frequently applied today because the primary source of the clinical event is in the follicular duct, not in the apocrine sweat glands.3 With increasing inflammation and severity of the disease process, the apocrine glands may become involved.

Prevalence estimates for hidradenitis suppurativa vary widely, from a low of 0.16% to a commonly accepted 1%. Some authorities cite an incidence of 4% in the United States.2,6,7 Recent large-scale self-report studies in France (n = 10,000) yielded a prevalence estimate of 1% in that population.8 Women are affected more often than men.3

Epidemiology and Genetics

Hidradenitis suppurativa follows a clinical pattern. First, hair follicles become occluded by keratin plugs. Next, inflammatory changes occur in the follicle. Cystic-appearing lesions develop, and bacteria colonize in these lesions. The lesions cause tissue damage and can produce significant scarring.1,2,7Apocrine glands are found in the areas of skin typically affected. Secondary inflammation develops in deep skin structures and results in apocrine gland inflammation, but apocrine glands are not involved in the primary pathologic process.3 While the clinical appearance is infectious, the condition is not, and skin-to-skin contact with others does not present a risk.

Some lesions may never completely resolve; they become chronic and produce abscesses. Patients with hidradenitis suppurativa may experience more than four inflamed lesions in a month, and the disease process may be active for more than 20 years.9 Organisms typically found in abscesses are Staphylococcus aureus, Streptococcus milleri, Staphylococcus epidermis, Staphylococcus hominis, Escherichia coli, Proteus mirabilis and Pseudomonas aeruginosa.2,3 Prior to abscess formation, lesions often yield sterile culture results.2,3 Family history of hidradenitis suppurativa is common in affected patients; a family history of severe nodulocystic acne may also exist.5,10 The literature reports a positive family history in 13% to 38% of patients with hidradenitis suppurativa, and there is disagreement about the possible inheritance pattern. Some literature describes an autosomal recessive pattern, which would be consistent with the lower end of the incidence spectrum (13%), but other research describes an autosomal dominant pattern, which is supported by the higher end of the observed inheritance spectrum (38%).1 An interesting genome scan in a Chinese family with a four-generation pedigree documenting hidradenitis suppurativa supports an autosomal dominant pattern.4

Management and Treatment Options

Hidradenitis suppurativa is a clinical diagnosis that is supported by history and clinical criteria. Factors in the history that support diagnosis include the following:2,8

  • onset after puberty
  • recurrence of lesions in typical sites over at least 6 months
  • poor or temporary response to antibiotic therapy
  • family history of similar lesions
  • current smoking or obesity.

The clinical presentation is of transient but recurrent inflammatory lesions. Some may have healed and produced significant scarring. The lesions may resemble cystic acne. The appearance suggests an infectious process, but the lesions are often sterile on culture, unless there has been progression to the abcess stage.3 The lesions are not contagious.2 Pain may consist of point tenderness only, or more diffuse tissue pain.11

hen symptoms are relatively mild (minimal pain, solitary or few nodules and no abcess formation), conservative medical treatment is appropriate. Self-care options include warm compresses or sitz baths, antibacterial soaps and topical antiseptics. Otherwise healthy patients should expect spontaneous improvement within 1 to 2 weeks of initiating these measures, although scarring may occur even in mild lesions.12,13

Incision and drainage is not recommended because it has not proven to affect the overall chronic, recurrent nature of the disease. In fact, incision and drainage may actually worsen local inflammation. Nonsteroidal anti-inflammatory agents can be used to manage any discomfort and to control inflammation.1-3 If lesions do not improve, patients should return for further evaluation.

Because hidradenitis suppurativa is typically a chronic condition, advise patients that when the lesions recur, they should promptly institute conservative medical measures to minimize tissue damage and discomfort. Advise patients who smoke or are overweight that tobacco use and obesity are aggravating factors for the disease process.2,8 Provide appropriate treatment recommendations to reduce these risks.

In moderate disease states, multiple nodules are present or recurrent, pain is moderate rather than mild, fluctuant superficial abcesses may have formed, and pronounced inflammation may lead to significant tissue damage. This inflammation appears to be triggered by an immune response, and referral to dermatology allows therapy with immunosuppressive agents such as etanercept (Enbrel), if appropriate.3,7 Referral to dermatology for photodynamic therapy is also recommended by some experts; this treatment uses a photosensitizing agent, molecular oxygen and light to selectively destroy cells.1

Fluctuant abscess formation makes incision and drainage more appropriate. This intervention may be especially helpful in areas where overlying tissues are thin. Stress that this provides short-term relief only and does not change the ongoing character of the disease process.2 Antibiotic therapy should be useful at this stage, with clindamycin (Cleocin) often recommended. Some authors encourage combining clindamycin with rifampin (Rifadin).3 Erythromycin and tetracycline are other recommended drugs.2

If tetracycline is used in women of reproductive age, effective contraception is essential. Low-androgen oral contraceptive formulations may provide serendipitous improvement in lesions.2 In fact, clinical trials evaluating the use of low-androgen oral contraceptives for hidradenitis suppurativa are under way.

Rifampin use may decrease the effectiveness of oral contraceptives. Advise patients on this therapy to use additional barrier methods as backup contraception.

Retinoids can be useful in the management of moderate stage hidradenitis suppurativa.1 Careful attention to effective contraception is also important for patients using this management regimen, due to the teratogenicity of this class of drugs.

Severe disease manifests with diffuse abcess formation in multiple sites, indurated skin with significant scarring, and possible formation of draining sinuses. Such cases require referral to a surgeon for acute treatment and to a dermatologist for ongoing care. Severe disease, especially in the perianal area in men, may merit biopsy to rule out malignancy.2 Aggressive wound care is important for severe presentations, with attention to minimizing exudate and removing slough. Cadexomer iodine gels and collagen matrix dressings containing silver have been used to achieve these goals.12 Referral to specialists in wound and ostomy care is important to provide patients with current evidence-based therapies.

For all patients with any level of disease burden, it is important to stress that the disorder is not associated with poor hygiene.2,12 Anecdotally, patients often assure their healthcare provider that they pay meticulous attention to skin hygiene, and in some cases they employ skin cleansing that is so vigorous it damages thin tissues in perineal areas. This is nonproductive and should be discouraged; routine, gentle skin cleansing with antibacterial soaps is preferred. The recurrent, chronic and sometimes debilitating nature of the disease increases the risk for depression, and regular screening for depression is advised.2,3,12

Putting It Into Practice

The nurse practitioner's holistic focus is invaluable in assisting patients who are coping with hidradenitis suppurativa. In addition to discomfort or pain, patients may experience significant disturbances in body image. This is especially true when lesions primarily affect the genitofemoral region, where the disease may adversely affect intimacy. It is helpful for patients who are experiencing feelings of isolation to be connected with others who share their diagnosis. One online resource for patients can be found at www.hs-usa.org. Patients who are interested in clinical trials for the treatment of hidradenitis suppurativa should visit www.clinicaltrials.gov.

Spontaneous remission may occur with aging, but recurrence should be expected, not regarded as a failure of treatment.2 When patients are aware of the high potential for recurrence, their feelings of loss of control may be lessened by the knowledge of self-care measures that can be initiated as soon as symptoms develop. Encourage patients to follow up promptly if no improvement occurs with conservative measures.

Kathleen Ahonen is a women's health nurse practitioner for the Monroe County Health Department in Monroe, Mich. She is an assistant professor of nursing at the University of Toledo College of Nursing in Toledo, Ohio.

References

1. Gold M. Aminolevulinic acid photodynamic therapy for hidradenitis suppurativa. Dermatol Clin. 2007;25(1):67-73.

2. Krbec A. Current understanding and management of hidradenitis suppurativa. J Am Acad Nurse Pract. 2007;19(5):228-234.

3. Kurzen H, et al. What causes hidradenitis suppurativa? Exp Dermatol. 2008;17(5):455-472.

4. Gao M, et al. Inversa acne (hidradenitis suppurativa): a case report and identification of the locus at chromosome 1p21.1-1q25.3. J Invest Dermatol. 2006;26(6):1302-1306.

5. Wolff K et al, eds. Fitzpatrick's Dermatology in General Medicine. 7thed. New York, N.Y.: McGraw-Hill; 734-738.

6. Herrington S. Hidradenitis suppurativa. In MR Dambro, ed. Griffith's 5 Minute Clinical Consult. 14thed. Philadelphia: Lippincott Williams & Wilkins; 2008: 570-572.

7. Hunger RE, et al. Toll-like receptor 2 is highly expressed in lesions of acne inversa and co-localizes with C-type lectin receptor. Brit J Dermatol. 2008;158(4):691-697.

8. Revuz JE. Prevalence and factors associated with hidradenitis suppurativa: results from two case-control studies. Am Acad Dermatol. 2008;59(4):596-601.

9. Von der Werth JM, Williams HC. The natural history of hidradenitis suppurativa. J Eur Acad Dermatol Venereol. 2000;14(5): 389-392.

10. Wiseman MC. Hidradenitis suppurativa: a review. Dermatol Ther. 2004;17(1):50-54.

11. Solarski ST. Living with hidradenitis suppurativa. Dermatol Nurs. 2004;16(5):447-449. Guidry MT. Use of cadexomer iodine gel and collagen matrix dressing in severe hidradenitis suppurativa. J Wound, Ostomy and Continence Nursing. 2008;35(3S):512.

12. Guidry MT. Use of cadexomer iodine gel and collagen matrix dressing in severe hidradenitis suppurativa. J Wound, Ostomy and Continence Nursing. 2008;35(3S):512.

13. Mayo Foundation for Medical Education & Research. Hidradenitis suppurativa. Medline Plus. Available at: http://www.nlm.nih.gov/medlineplus/hidradenitissup purativa.html. March 3, 2010.




     

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