Hidradenitis suppurativa: a treatment challenge - american family physician

Hidradenitis Suppurativa:
A Treatment Challenge
NIPA SHAH, M.D., University of Illinois College of Medicine, Chicago, Illinois
Hidradenitis suppurativa is a chronic, recurrent, debilitating disease that presents with painful,
inflamed lesions in the apocrine-gland–bearing areas of the body, most commonly the axillary,
inguinal, and anogenital areas. Etiology traditionally has been attributed to occlusion of the
apocrine duct by a keratinous plug; however, defects of the follicular epithelium also have been
noted. Contributing factors include friction from axillary adiposity, sweat, heat, stress, tight
clothing, and genetic and hormonal components. Multiple treatment regimens are available,
including antibiotics, retinoids, corticosteroids, incision and drainage, local wound care, local
excision, radiation, and laser therapy. However, no single treatment has proved effective for all
patients. Radical excision of the defective tissue is the most definitive treatment. The psycho-
logical impact on the patient can be great, encompassing social, personal, and occupational
challenges. This impact should be addressed in all patients with significant disease. (Am Fam
Physician 2005;72:1547-52, 1554. Copyright 2005 American Academy of Family Physicians.)
S Patient information:
A handout on hidradenitis suppurativa, written by the author of this article, is provided on page 1554.
Hidradenitis suppurativa (from Diagnosis
the Greek hidros, sweat, and The clinical presentation of hidradenitis aden, glands), is also known suppurativa indicates the diagnosis. A thor-as Verneuil’s disease or acne ough history and physical examination inversa, and occasionally is spelled hydrad- are recommended at the initial visit. Early enitis. It is a common disorder, but its exact symptoms may include discomfort, itch-prevalence in the United States is unknown. ing, erythema, burning, and hyperhidrosis. A Danish study1 noted a prevalence of Hidradenitis manifests most commonly as 4 percent in women. However, the diagnosis tender, nodular lesions in the axillae (Figure of hidradenitis suppurativa often is over- 1), although other parts of the body also looked by physicians and therefore may be may be affected (Table 1 and Figure 2). If a more common than is recognized. Hidrad- single nodule appears, it may indicate one enitis suppurativa affects more women than of several other skin lesions that manifest men, with a female-to-male predominance as high as 4:1.2 This painful, disfiguring, and at times debilitating disease is marked sional secondary infection, and intermittent remissions that can last several years. The disease almost always occurs after puberty and before age 40, leading to the theory that there is a hormonal component to the pathogenesis. Flare-ups have been linked with menses3; shorter menstrual cycles and longer duration of menstrual flow are asso-ciated with the disease.1 There also seems to be a genetic component, and in one study3 Figure 1.
of 110 patients, 38 percent reported a fam-ily history of this disease. This may reflect a familial form with autosomal dominant inheritance.4 October 15, 2005 U Volume 72, Number 8 American Family Physician 1547
SORT: KEY RECOMMENDATIONS FOR PRACTICE
ated with several other conditions (Table 3).8,9 In perianal hidradenitis, biopsies should be Early, rather than delayed, wide excisional performed to exclude the possibility of coex- isting cancer.10 Crohn’s disease should also be disease severity allows for more surgical A = consistent, good-quality patient-oriented evidence; B = inconsistent or limited- Complications
quality patient-oriented evidence; C = consensus, disease-oriented evidence, usual Potential complications of hidradenitis sup- practice, expert opinion, or case series. For information about the SORT evidence rating system, see page 1435 or http://www.aafp.org/afpsort.xml. purativa include dermal contraction, local or systemic infection resulting from the spread of microorganisms, arthritis second- in a similar fashion, and distinguishing ary to inflammatory injury, squamous cell hidradenitis can be difficult. A differential carcinoma (in indolent sinus tracts), dis-diagnosis is given in Table 2.5-7 Nodules may seminated infection (rare), restricted limb have malodorous, superinfected drainage. mobility from scarring, lymphedema caused Rarely, the patient has a fever or is septic, by lymphatic injury from inflammation and or both. In these instances, further work-up scarring, rectal or urethral fistulas, sys- is based on laboratory findings. Complete temic amyloidosis, and anemia from chronic blood count, blood cultures, and routine infection.11 chemistries should be considered. Culture of the drainage is a reasonable option to help direct treatment.
As the disease progresses, the diagnosis Areas of the Body Affected
becomes more apparent, especially if the by Hidradenitis Suppurativa
patient presents with frequent recurrences, scarring, fistulous tracks, and incom- plete healing. The clinical course varies from occasional axillary lesions to diffuse abscess formations in multiple sites lead- ing to chronic draining sinuses, as well as indurated, scarred skin and subcutaneous tissues. Some areas may coalesce to form tender, raised, violaceous dermis (Figure 3).
TABLE 2
Differential Diagnosis
for Hidradenitis Suppurativa
The rightsholder did not grant rights to reproduce Figure 2.
Information from references 5 through 7. 1548 American Family Physician
Volume 72, Number 8 U October 15, 2005 Hidradenitis Suppurativa
Treatment
There is no cure for hidradenitis suppurativa. However, several treatment options are avail-able, including preventive, medical, surgi- cal, and psychological strategies (Table 4).
Because of the variety of ways in which the disease can manifest and progress, treatment should be based on the patient’s presentation and circumstances. Few high-quality ran- domized controlled trials or systemic reviews have addressed treatment of this condition.
advice for the prevention of hidradenitis suppurativa exists, few methods have proved Figure 3.
to be effective for all patients. In one study,324 percent of patients found nothing to help their condition, despite an average disease duration of almost 19 years.
Conditions Associated
with Hidradenitis Suppurativa
and depilation have not been supported as a cause for this discrepancy in at least one study.12 However, these should be avoided if they cause irritation. Warm compresses, topical antiseptics, and antibacterial soap may help in patients with folliculitis. To help alleviate patient anxiety about the condition, physicians should emphasize that hidradeni- tis suppurativa is not caused by poor hygiene advanced by excessive underarm adiposity because this creates an ideal environment for Information from references 8 and 9. bacterial growth and also produces friction. Therefore, one method of prevention may be weight loss. However, although losing as warm baths, hydrotherapy, and topical weight improves symptoms, it does not pro- cleansing agents to reduce bacterial load.13 vide a cure. Friction from clothing increases Nonsteroidal anti-inflammatory drugs may pain and discomfort, and patients should alleviate pain as well as inflammation. Anti-avoid wearing tight, synthetic clothing near biotics, although not proven to be effec-the affected areas. Heat and humidity also tive, are the mainstay of medical treatment, have been associated with flare-ups, and especially for lesions suspected of being prolonged exposure to hot, humid climates superinfected. should be avoided if possible. Stress man- There is no evidence that chronic suppress- agement methods may be useful because the ive antibiotic therapy alters the natural his- disease can be aggravated during times of tory of hidradenitis. In a study3 of 110 patients increased psychosocial stress.
with hidradenitis, the average duration of painful nodules was 6.9 days—about the dura- MEDICAL TREATMENT
tion of an average course of antibiotics. There- Initial treatment of hidradenitis suppurativa fore, the perceived response of hidradenitis can begin with conservative measures such suppurativa to antibiotics may be explained October 15, 2005 U Volume 72, Number 8 American Family Physician 1549
TABLE 4
Treatment of Patients with Hidradenitis Suppurativa
Avoidance of prolonged exposure to heat and humidity Avoidance of shaving if irritation occurs Avoidance of tight, synthetic clothing near affected area Topical antiseptics and antibacterial soap Warm compresses, warm baths, and hydrotherapy NOT RECOMMENDED: Use of simple incision and drainage for lesions that are not fluctuant abscesses with purulent discharge Antibiotics (topical, systemic, or both): Cephalosporins (if patient has concurrent cellulitis) Oral contraceptive agents, with high estrogen-to-progesterone ratio and low androgenicity of progesterone, in selected women CONSIDER: Referral to a dermatologist (for patients who do not respond to initial therapy) or an early referral to an experienced general surgeon (discuss risks and benefits of surgery) Diffuse abscess formation in multiple sites; chronic draining sinuses; indurated scarred skin and subcutaneous tissues NOTE: All patients also should be offered reassurance and psychosocial support. by the natural history of the condition itself, Empiric antibiotic treatment may be given calling into question the routine use of anti- biotics.3 One option is to culture the drainage days’ observation have not improved symp- from a large nodule and treat based on the toms. However, when superinfection is sus-results. Staphylococcus commonly is isolated; pected it is best to treat based on culture other pathogens include Escherichia coli and results of drainage. Treatment can begin H-hemolytic streptococcus. Enteric flora may with topical or systemic antibiotics, or both. be found in cultures from perianal regions. The only topical antibiotic that has been Multiple organisms, including anaerobic bac- proven effective in a randomized controlled trial is clindamycin (Cleocin).1 Antistaphy-lococcal agents are best for axillary dis-ease, and more broad-spectrum coverage The Author
is better for perineal disease. Dicloxacillin (Dynapen; 1 to 2 g daily), erythromycin NIPA SHAH, M.D., is director of predoctoral education at the University of Illinois College of Medicine, Chicago, where she received her medical degree. She com- (1 g daily), tetracycline (1 g daily), and mino- pleted a family medicine residency at the University of New Mexico Department cycline (Minocin; 1 g daily) have been used. of Family and Community Medicine, Albuquerque.
Cephalosporins may be helpful for concur-rent active cellulitis. For severe, recurrent Address correspondence to Nipa Shah, M.D., University of Illinois, Department of Family Medicine, 1919 West Taylor, M/C 663, Chicago, IL 60612 (e-mail: disease, anecdotal evidence suggests that [email protected]). Reprints are not available from the author. two months or more of antibiotic therapy 1550 American Family Physician
Volume 72, Number 8 U October 15, 2005 Hidradenitis Suppurativa
may be needed to prevent progression and experts as the treatment of choice because worsening of concomitant infection.
repeated failed treatments lead to the dis- Other medical treatment options address ease being more widespread and severe at the possible hormonal etiology of hidradeni- presentation, making surgical options more tis; these options include oral contraceptive difficult.17,20 Patients should be agents that contain a high estrogen-to- Obesity, insufficient
progesterone ratio and low androgenicity the disease that is present at the excision, significant skin
of progesterone.14 Two patients with severe, site of the excision; recurrence maceration, and chronic
long-standing disease benefited from finas- skin infection may increase
teride (Proscar) at a dosage of 5 mg daily.15 In the incidence of recurrence.
Europe, the antiandrogen cyproterone ace- tate (Cyprostat) has been successful in some studies,16 but it currently is not approved for anal disease, 3 percent for axillary disease, and 37 percent for inguinoperineal disease. Oral retinoids, which work by inhibiting Obesity, insufficient excision, significant skin sebaceous gland function and abnormal maceration, and chronic skin infection may keratinization, also have been used. Pre- increase the incidence of recurrence.21 In treatment with isotretinoin (Accutane) at another study,20 the overall complication rate a dosage of 0.5 to 1.0 mg per kg daily for a was 17.8 percent; most complications were few months before surgery has been recom- minor, such as suture dehiscence, postop- erative bleeding, and hematoma. The rate of ponents.17 No consensus on the dosing and recurrence in this study was 2.5 percent and duration of isotretinoin therapy has been was related to the severity of the disorder.20reached by the few studies that have investi-gated it as a possible therapeutic option. Side OTHER TREATMENT OPTIONS
effects of isotretinoin remain a major issue Radiotherapy has been investigated as a and include birth defects, hepatotoxicity, potential treatment option. In a study22 of pseudotumor cerebri, and aggression.
the effects of radiotherapy in 231 patients, Corticosteroids and immunosuppressants 38 percent had complete relief, and 40 per- are other treatment possibilities. Topical tri- amcinolone (Aristocort) may be an option, toms. However, the possibility of long-term but insufficient research has been conducted side effects must be discussed thoroughly for it to be recommended routinely. Oral with the patient. cyclosporine (Sandimmune) has shown some benefit, but chronic treatment can In one small study,23 10 patients who did cause serious toxicity.18 not respond to systemic antibiotics were given one cycle of cryotherapy; eight patients SURGICAL TREATMENT
For early, limited disease that presents with a also experienced significant pain, prolonged fluctuant abscess, incision and drainage may healing time (average, 25 days), and post- be a good first option. However, this proce- treatment infection. A carbon dioxide laser dure provides only short-term relief and has used in conjunction with second-intention little impact on the disease course. When healing provided relief for a few patients.24
hidradenitis sinus tracks are well estab-
lished but relatively superficial, they can be Counseling
unroofed or laid open.19 Because these tracks
In addition to treating the physical illness, are lined by epithelium, the floor of the it is crucial that physicians acknowledge track can be preserved; this facilitates rapid and treat the psychological burden associ-healing and minimizes scarring.10 ated with the disease. Because of the areas of Early, rather than delayed, wide excisional the body that are affected, the malodorous therapy has been recommended by some discharge, the chronic discomfort, and the October 15, 2005 U Volume 72, Number 8 American Family Physician 1551
Hidradenitis Suppurativa
general unsightliness of the disease, hidrad- tiva. The Lahey Clinic experience. Dis Colon Rectum 1990;33:731-4.
enitis suppurativa poses many challenges for 6. Church JM, Fazio VW, Lavery IC, Oakley JR, Milsom JW. patients in their personal life. Sexuality can The differential diagnosis and comorbidity of hidrad- be negatively affected. Unforgiving societal enitis suppurativa and perianal Crohn’s disease. Int J attitudes regarding inappropriate body odor (especially for those who choose not to wear 7. Fite, Diana. Hidradenitis suppurativa. Emedicine 2001. Accessed online June 13, 2005 at: http://www.emedi- deodorants), as well as years of inadequate treatment, may lead to frustration, depres- 8. Roberts JR, Hedges JR, eds. Clinical procedures in emer- sion, and isolation. For patients at increased gency medicine. 3d ed. Philadelphia: WB Saunders, 1998.
risk for these outcomes, early surgical inter- 9. Barth JH, Ng LL, Wojnarowska F, Dawber RP. Acanthosis nigricans, insulin resistance and cutaneous virilism. Br J Dermatol 1988;118:613-9.
Final Comment
10. Townsend CM, Sabiston DC. Sabiston Textbook of sur- gery: the biological basis of modern surgical practice. 16th ed. Philadelphia: WB Saunders, 2001.
lenging disease for patients and physicians. 11. Jansen I, Altmeyer P, Piewig G. Acne inversa (alias Because there has been no significant research hidradenitis suppurativa). J Eur Acad Dermatol Venereol comparing treatment options, the choice of therapy should depend on the patient’s cir- 12. Morgan WP, Leicester G. The role of depilation and deodorants in hidradenitis suppurativa. Arch Dermatol cumstances and preferences, the outcome of previous treatments, the experience of the 13. Paletta C, Jurkiewicz MJ. Hidradenitis suppurativa. Clin physician, local expertise (e.g., a surgeon or dermatologist who specializes in treatment of 14. Behrman RE, Kliegman R, Jenson HB, eds. Nelson Text- book of pediatrics. 16th ed. Philadelphia: WB Saunders, the disease), and the chronicity and severity at presentation. Further research should be con- 15. Farrell AM, Randall VA, Vafaee T, Dawber RP. Finas- ducted not only on the etiology of this disease teride as a therapy for hidradenitis suppurativa. Br J but also on the optimal treatment regimen.
16. Mortimer PS, Dawber RP, Gales MA, Moore RA. A The author thanks Patrick Tranmer, M.D., M.P.H., for help double-blind controlled cross-over trial of cyproterone acetate in females with hidradenitis suppurativa. Br J Dermatol 1986;115:263-8.
Members of various family medicine departments 17. Jansen T, Plewig G. Acne inversa. Int J Dermatol, develop articles for “Practical Therapeutics.” This article is one in a series coordinated by the Department of 18. Gupta AK, Ellis CN, Nickoloff BJ, Goldfarb MT, Ho Family Practice at the University of Illinois College of VC, Rocher LL. Oral cyclosporine in the treatment of Medicine at Chicago–Rockford. Coordinator of the series inflammatory and noninflammatory dermatoses. A clinical and immunopathologic analysis. Arch Dermatol 1990;126:339-50.
Author disclosure: Nothing to disclose.
19. Culp CE. Chronic hidradenitis suppurativa of the anal canal. A surgical skin disease. Dis Colon Rectum REFERENCES
20. Rompel R, Petres J. Long-term results of wide surgical 1. Jemec GB. The symptomatology of hidradenitis sup- excision in 106 patients with hidradenitis suppurativa. purativa in women. Br J Dermatol 1988;119:345-50.
2. Galen WK, Cohen I, Roger M, Smith MH. Bacterial 21. Harrison BJ, Kumar S, Read GF, Edwards CA, Scanlon infections. In: Schachner LA, Hansen RC, eds. Pediatric MF, Hughes LE. Hidradenitis suppurativa: evidence for dermatology. 2d ed. New York: Churchill Livingstone, an endocrine abnormality. Br J Surg 1985;72:1002-4.
22. Frohlich D, Baaske D, Glatzel M. Radiotherapy of 3. Von der Werth JM, Williams HC. The natural history of hidradenitis suppurativa—still valid today? Stranlenther hidradenitis suppurativa. J Eur Acad Dermatol Venereol 23. Bong JL, Shalders K, Saihan E. Treatment of persistent 4. Von der Werth JM, Williams HC, Raeburn JA. The clini- painful nodules of hidradenitis suppurativa with cryo- cal genetics of hidradenitis suppurativa revisited. Br J therapy. Clin Exp Dermatol 2003;28:241-4.
24. Finley EM, Ratz JL. Treatment of hidradenitis suppurativa 5. Wiltz O, Schoetz DJ Jr, Murray JJ, Roberts PL, Coller with carbon dioxide laser excision and second-intention JA, Veidenheimer MC. Perianal hidradenitis suppura- healing. J Am Acad Dermatol 1996;34:465-9.
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