Hidradenitis suppurativa (HS) is a chronic and relapsing condition occurring in areas that contain apocrine sweat glands, usually the axillae and groin. This disease is sometimes associated with severe cystic acne and pilonidal sinuses (follicular occlusion syndrome). Comedone-like follicular occlusion, chronic relapsing inflammation, mucopurulent discharge, and progressive scarring characterize HS lesions.1-6
HS affects more women than men, and it can be very painful and disfiguring. NPs should be familiar with HS and understand the diagnostic process and treatments to provide the best outcomes for patients.
PREVALENCE AND PATHOPHYSIOLOGY
HS symptoms were first described in 1839 by Velpeau when a patient presented with superficial abscesses in the axillary, breast, and perianal regions. In 1854, Verneuil associated the condition with the apocrine glands, and the condition was given its current name, although for many years, the process was referred to as Verneuil disease.6,7
The prevalence of HS is approximately 1% to 2% of the general population.6,8 Although it is seen in individuals worldwide, hot, humid environments tend to support its development. HS affects all races; however, severe cases are most often seen in people with black skin, possibly because they may have more apocrine glands than other races.4,6 Males have a higher incidence of anogenital involvement, whereas females experience higher axillary involvement. HS can present anytime between puberty and postmenopause, and the onset of HS peaks in individuals between the ages of 11 and 50 years old.
Predisposing factors include obesity, smoking, genetic predisposition to acne, apocrine duct obstruction, and secondary bacterial infection.3,5 Because apocrine glandsare stimulated by androgen and suppressed by estrogen, symptoms are often worse during the menstrual period and decrease after menopause. This may also explain the common findings of hirsutism and obesity in affected women.6,9-11
Heredity may play a role in the predisposition to HS but there have not been any studies to definitively support heredity as a predisposing factor. While there is no data available to support this, there have been affected mother-daughter dyads noted.5-7 Chemical irritants, such as deodorants or antiperspirants, and mechanical irritation, such as shaving or depilatory use, may also be risk factors.6-8,12 Despite the severity of disfigurement and infection associated with HS, the death rate is similar to that of the general adult population.5,6,8
There is ongoing debate about the etiology of HS. The classic view is that HS is an occlusive and infectious disease of the apocrine glands. In more recent studies, however, HS is identified as a disorder of follicular occlusion rather than apocrine occlusion.5,6,10,13
Onset of HS is insidious and the severity of symptoms varies widely. (See HS fact sheet.) Early symptoms of HS include pruritus, erythema, and local hyperhidrosis. Later, the lesions become painful, purulent, and disfiguring.4,6
Primary lesions can be painful or tender erythematous papules or nodules, painful or tender abscesses and inflamed draining papules or nodules, dermal contractures and ropelike elevation of the skin, or double-ended (bridged) comedones.4,6
Areas that may be affected by HS lesions are the axillae, areola of the breast, submammary fold, periumbilical region, groin, scalp, zygomatic areas of the face, nape of the neck, external auditory meatus, and shoulders.6,10
Most patients will present with a firm, pea-size nodule that may spontaneously rupture and have purulent discharge. The lesion then heals with fibrosis and another can occur in an adjacent area. The progression from noninflamed nodules to painful, round, deep-seated lesions and subsequent scarring is highly diagnostic. Characteristic progression of lesions are physical findings used by NPs to diagnose HS. The nodules tend to coalesce and may become infected, resulting in acute abscesses. These abscesses may temporarily resolve or may progress to multiple abscesses or draining sinuses with persistent pain, fistula formation, and scarring and subsequent restricted mobility. Bridged comedones are the hallmark finding of HS.6
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Many dermatologic conditions share several symptoms of HS. Some conditions to consider are actinomycosis, catscratch disease, erysipelas, granuloma inguinale (donovanosis), lymphogranulomavenereum, infected Bartholin cyst, carbuncle, lymphadenitis, tuberculosis, and tularemia.4,6,10 HS may present in association with other conditions as well, including Crohn disease, irritable bowel syndrome, Down syndrome, certain forms of arthritis, Gravesdisease, and Sjogren syndrome. Other differential diagnoses to consider are bacterial folliculitis, pilonidal cysts, and furunculosis.4,6,10
Diagnosis of HS is primarily clinical, and biopsy is rarely required. Practitioners must consider three elements before making a diagnosis: typical lesions, characteristic distribution of lesions, and recurrence.4,6,10
To make a diagnosis of HS, the patient must have one of the following6:
* active disease with one or more primary lesions in a designated site (axillae or groin), plus a history of three or more discharging or painful lumps (abscesses) in designated sites since puberty
* inactive disease with a history of five or more draining or painful abscess-like lumps in designated sites since onset of puberty, in the absence of current primary lesions.
The disease has three clinical stages. The characteristics of the first stage include a few isolated lesions without scarring or tracts. Long periods of remission may delay an immediate, definitive diagnosis of HS.6
The second stage involves recurrent abscesses, or single or multiple widely separated lesions with sinus tracts and scarring. Remissions are rare at this stage. Most diagnoses of HS occur at this stage, and referral to surgery is often indicated.6
The third, most devastating clinical stage is characterized by diffuse, broad involvement with multiple interconnecting sinus tracts and abscesses across a broad area of the body. At this stage, scarring and oozing lesions are common. Because remission is unlikely, surgery is most often recommended, although other treatments may be considered and tried.6
Lab studies may be helpful in evaluating for HS. These include complete blood cell (CBC) count with differential, platelet count, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) assay, urinalysis, routine comprehensive metabolic panel, thyroid, and anemia workups. Blood cultures may be necessary if the patient appears septic. Although the typical bacteria involved are usually staphylococciand streptococci, cultures of the exudates can ensureappropriate antibiotic therapy.4,11
Local hygiene with ordinary soap, weight reduction, use of antiseptics, warm compresses, and loose-fitting clothes are among the recommendations for initial treatment.4,6,10 Silver-impregnated and charcoal-impregnated dressings may be helpful with antimicrobial action and odor, respectively.14 Absorptive quality in a dressing is important as well.
Oral antibiotics are the initial treatment of choice. In mild cases, oral antibiotics may help suppress inflammation. The antibiotics used for acne are appropriately used in higher doses for HS treatment. These include erythromycin (E-mycin), tetracycline (Sumycin), minocycline (Minocin), and doxycycline (Vibramycin).4,7,10 Intralesional injections of corticosteroids (triamcinolone injections) are used for early stages of HS. Nonsteroidal anti-inflammatory drugs (NSAIDs) are also useful to manage the pain associated with HS.4,7,10,12 Use of silver-impregnated alginates may help in reducing bacterial load as well as managing drainage.
Tumor necrosis factor (TNF) blockers appear to be effective for HS management. Infliximab (Remicade) has been noted for off-label treatment for HS. There is evidence that off-label use of adalimumab (Humira) may also be helpful for treating HS (this drug inhibits TNF-alpha). Although the studies were small and require further investigation, results showed improved quality of life with the use of TNF blockers.10,15-20
Most nodules resolve without being drained; however, incision and drainage is recommended if nodules are very large, fluctuant, and painful. The nodules often track or tunnel beneath the skin in a complex, web-like pattern, leavingscar tissue where healing has occurred.21,22
Radical surgery, although aggressive, is the most effective, curative treatment for late-stage hidradenitis. To be successful, the entire area of affected, scarred tissue must be removed. This may necessitate skin grafting once the area is completely excised. Carbon dioxide laser has been proposed as a better alternative to remove glandular tissue, and the wound is left to close by secondary intention. Vacuum-assisted closure has been used to promote healing after radical excision of HS lesions.22,23
Plastic and reconstructive surgical referral and collaboration with infectious disease specialists are warranted in HS cases. Also, patients may need referral to an immunologist to help identify any underlying immune-mediated disease processes.21
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HS is a painful and disfiguring disease that unfortunately may not be identified until later in the process. Because of this delay, HS may be more common than once thought. Treatment options are limited and not considered curative. The NP may also further assist the patient by providing resources for support and information about the disease. These resources may empower the patient with information that will enhance his or her ability to be active and educated participants during the treatment process.