Shannon Rylee, M.D. and Nasim Afsarmanesh, M.D.
A 35-year-old woman with polycystic ovarian syndrome and hidradenitis suppurativa presented to the Emergency Department with vulvar and axillary lesions with increasing pain and drainage. She was diagnosed with HS nine years prior and was undergoing treatment with isotretinoin after treatment failure with adalimumab and etanercept. Prior surgical treatment of lesions failed to provide any relief.
On physical examination, she had multiple, exquisitely tender abscesses on perineal and axillary areas that were freely draining pustular material (Figure 1). Computed tomography of the pelvis revealed subcutaneous lesions connecting to the skin surface with associated inguinal lymphadenopathy. Wound cultures were polymicrobial growing Staphylococcus, Corynebacterium, Proteus, and Bacteroides. She was treated for abscesses with metronidazole, ceftazidime, and vancomycin. General surgery was consulted and performed incision and drainage of vulvar lesions. Drainage and tenderness of lesions improved significantly. She was discharged home on amoxicillin clavulanate and ciprofloxacin.
Hidradenitis suppurativa (HS) is a follicular occlusive disease, which often causes severe scarring of skin leading to fistula formation with draining sinuses. While pathogenesis is unknown, it is hypothesized that terminal follicular obstruction leads to damage of follicles with associated chronic inflammation and fibrosis of apocrine and eccrine glands. The incidence of HS is approximately 1% of the general population and affects women more frequently than men. Onset of symptoms is typically after puberty, but before age 40 years.1 Typically, patients experience 2 to 4 new lesions per month, each lasting approximately 7 days. However, nearly two-thirds of patients at all stages of the disease report at least one permanent lesion. Predisposing factors may include obesity, hyperandrogenism, and possible genetic factors. It has been associated with smoking, certain oral contraceptive pills, and lithium. Cultures from non-infected lesions are often sterile, indicating that infection and skin colonization with bacteria are unlikely the inciting etiology. Lesions are independent of personal hygiene.2 Complications of long-standing disease can include fistula formation into other epithelial lined organs, lymphedema, hypoproteinemia, anemia, and depression. Frequently, HS remits after menopause.
Diagnosis of HS is clinical and does not require biopsy. For diagnosis, typical lesions, as seen in Figure 1, must occur in characteristic sites, axilla and inguinal regions. They must also follow a relapsing and chronic course. Lesions are classified as primary, secondary, or tertiary. Primary lesions are first to appear in characteristic locations. Secondary lesions recur at the same site, often forming sinus tracts. Tertiary lesions cause extensive scarring and interconnected sinuses. Unfortunately, this is typically the stage at which the disease is diagnosed.
Management is dictated by disease severity according to Hurley's staging. Stage I includes abscess formation without sinus tracts or fibrosis and medical therapy is recommended. Stage II has recurrent abscesses with sinus tract formation with fibrosis. Drug therapy is preferred with only limited excision of certain lesions. Stage III is diffuse disease with extensive fibrosis, abscesses and sinus tract formation for which wide surgical excision is recommended.
Figure 1. Hidradenitis suppurativa: axillary lesions
There are no large clinical trials for management of HS. Recommendations for all patients include avoiding trauma to the affected area, losing weight, smoking cessation, using gentle antibacterial soaps, and avoiding shaving these regions. Antibiotics are used for secondary infections, but they do not alter the primary course of disease. Antiandrogens may be useful; however, in trials, only finasteride has provided minimal relief. Clinical response with retinoids is variable, although it has been helpful perioperatively. Immunosuppressive medications have had the most beneficial effects. Infliximab has been used with some success for severe Stage III HS. However, it does not alter sinus tract formation.3
There are a variety of surgical interventions available, including incision and drainage, unroofing procedures and excision. Incision and drainage are performed for pain relief. Concurrent oral antibiotics are recommended. Unroofing treatments are the preferred intervention for extensive HS. It involves mapping all sinus tracts, opening them with scissors, and then removing the entire roof of the tract. Wide excision could be used for the most severe cases, although this procedure has a recurrence rate of 50% when used with primary closure. In general, primary closure of the wound is not recommended, as it buries remaining foci of active disease.4
Hidradenitis suppurativa can spontaneously regress and go into remission, although this is often after the disease has left terrible scars, both physically and emotionally.5 However, cure is unlikely if the disease is widespread. Unfortunately, up to 25% of patients have reported no treatment for management of their disease.
This case illustrates a unique condition with high prevalence in the general population. Immediate recognition and diagnosis can avoid unnecessary antibiotic treatment, while expediting appropriate counseling and therapy.
Submitted on October 14, 2008
© copyright 2014 Anonymous & UCLA Department of Medicine