In the latest article in our Clinical Practice review series, Hidradenitis Suppurativa, a 50-year-old woman presented with fatigue and shortness of breath. Dyspnea after moderate exertion had developed gradually, along with profound malaise and a nonproductive cough. In the 48 hours before admission, her shortness of breath had worsened.
Hidradenitis suppurativa is a chronic, recurrent inflammatory disease affecting skin that bears apocrine glands. It usually develops after puberty, manifested as painful, deep-seated, inflamed lesions, including nodules, sinus tracts, and abscesses. In most patients, flares are accompanied by increased pain and suppuration at varying intervals, often occurring premenstrually in women. If untreated, the flares subside within 7 to 10 days.
• What are the risk factors associated with development of hidradenitis suppurativa?
Women are more frequently affected than men (female:male ratio, 2:1 to 5:1) and appear to be more likely to have genitofemoral lesions. The condition most commonly develops in persons in their early 20s, although the onset has been described in prepubertal children and in postmenopausal women as well. The prevalence of the disease appears to decline at an age of more than 50 years. About one third of patients with hidradenitis suppurativa report a family history of the disease, and affected families with an autosomal dominant mode of inheritance have been identified. Cigarette smoking is a recognized risk factor for both the development of hidradenitis suppurativa and severe disease. Obesity is also a risk factor; the majority of patients are overweight, and both body-mass index and tobacco smoking have been directly correlated with the severity of this condition.
• What conditions are associated with hidradenitis suppurativa?
The frequency of hidradenitis suppurativa has been reported to be increased among patients with Crohn’s disease, affecting 17% of such patients, according to one report. A relationship between the two conditions is supported by clinical, histologic, and epidemiologic similarities, such as sinus tracts, granulomatous inflammation, scarring, and onset after puberty. Arthritis (rheumatoid factor-negative and HLA-B27-negative) is also more frequent among patients with hidradenitis suppurativa than in the general population and usually involves the peripheral joints, often in an asymmetric manner. Data from an epidemiologic study suggested a 50% increase in the risk of cancer of any kind in patients with hidradenitis suppurativa, as compared with the general population; specific cancers reported to occur more frequently in these patients included squamous-cell carcinoma, buccal cancer, and hepatocellular cancer. However, this study did not adjust for status with respect to cigarette smoking, and it is likely that the observed associations were confounded by smoking — a recognized risk factor for both hidradenitis suppurativa and these diseases.
Morning Report Questions
Q: How is hidradenitis suppurativa diagnosed?
A: The diagnosis of hidradenitis suppurativa is generally made clinically. On physical examination, one may see characteristic inflamed and noninflamed nodules; draining and nondraining sinus tracts; and abscesses in the axillary, inguinal, and anogenital regions. The lesions occasionally extend beyond these areas and appear around the anus, on the buttock, or on the breast in females. The nodules are located in the deeper dermis and are rounded rather than having the pointed, purulent appearance of simple boils. Secondary lesions such as pyogenic granulomas in sinus tract openings, plaquelike induration, ropelike scars, and giant multiheaded comedones may also be found. In selected cases, additional testing may be helpful. Biopsies and bacterial cultures are indicated only in atypical or refractory cases. Routine bacteriologic studies of the lesions in hidradenitis suppurativa are most frequently negative, although flares may be associated with superinfection involving a range of bacteria, including Staphylococcus aureus. If extensive surgery is planned, ultrasonography may help in the preoperative assessment by identifying subclinical extension of the lesions.
Q: How is hidradenitis suppurativa treated?
A: Stage I (localized) disease is usually managed with topical therapy, whereas systemic therapy is recommended for more widespread or severe disease. Topical administration of clindamycin (10 mg per milliliter twice daily) for 3 months has been found to reduce the number of abscesses, nodules, and pustules. Intralesional injections of glucocorticoids (e.g., triamcinolone, 2 to 5 mg) are sometimes helpful for individual lesions. When topical treatment is insufficient, oral antibiotics (often those with antiinflammatory properties) are commonly used. Tetracycline at a dose of 500 mg twice a day is often utilized, though it is unclear that it is superior to topical therapy. Combination antibiotic therapy with clindamycin and rifampicin (each typically given at a dose of 300 mg twice daily) has demonstrated benefit. Antiandrogens are sometimes used. Case series have suggested a lack of benefit from isotretinoin. In severe cases of hidradenitis suppurativa, systemic immunosuppressive agents have been used, such as cyclosporine on TNF-(alpha) inhibitors. In cases of individual scarred lesions or more severe disease, surgical options offer the best chances of cure. Incision with drainage is discouraged, since recurrence is the norm.