Hidradenitis Suppurativa (HS)

Introduction

hidradenitis image
Figure X1. Area affected by Hidradenitis suppurativa

Hidradenitis Suppurativa (HS) is an uncommon cutaneous condition, which primarily affects young individuals and exhibits chronicity with frequent flare-ups followed by quiescent periods. The severity of this disease is variable. It may present initially as an abscess, but is typically chronic or multiply recurrent in the affected area, and ultimately can lead to severe scarring, contracture, possible malignancy, and disability for the patient.

Hidradenitis suppurativa commonly occurs in the perineum, axilla, and groin, but can also be seen in the inguinal and mammary regions as well. These areas contain high concentration of apocrine glands, which may play a role in the disease.

Incidence and Aetiology

The exact incidence of hidradenitis suppurativa is not known; however, 1 in every 300 individuals may be affected in some way. African Americans appear to be affected more often than Caucasians, and perianal disease is approximately twice as common in males.

Almost all patients present after puberty in the second and third decades of life, thereby implicating hormones and the development of secondary sexual characteristics as potential causative factors.

Harrison et al. demonstrated androgen excess and a decrease in progesterone in patients with hidradenitis. Obesity has been suggested as a predisposing factor through increased shear forces in the affected areas; however, it is more likely an aggravating factor rather than a causative factor.

Additional predisposing factors include tobacco use, acne, stress, poor skin hygiene, excessive heat, hyperhidrosis, and chemical depilatories. In a series from the Ochner clinic, 70% of affected patients were smokers, but no causal relationship could be definitively shown.

Proposed mechanism of tobacco influence suggests that smoking may alter granulocytes, modify sweat gland activity, and give off toxic metabolites in sweat.

Perianal hidradenitis affects males twice as often as females, but hidradenitis in all locations may be more common in females and African-American persons. Fortunately, for sufferers of perianal hidradenitis, it appears to recur less often after surgical treatment (<0.05%) than does inguinal-perineal disease (37–74%).

Bacteriology

Wound cultures from hidradenitis patients have grown Staphylococcus epidermidis, Escherichia coli, Klebsiella, Proteus, α-Streptococcus, anaerobic bacteria, and diphtheroids; however, one study reported that wound cultures of early lesions were negative.

S. epidermidis and Staphylococcus aureus are the most frequently isolated organisms from hidradenitis supprativa lesions. Chlamydia trachomatis, often associated with lymphogranuloma venereum, and Bilophila wadsworthia infection have also been implicated in hidradenitis, but the clinical significance is not known.

Pathophysiology

It is believed that hidradenitis suppurativa originates from the occlusion of hair follicles.

Dilation and rupture of hair follicles into the dermis leads to dermal infiltration by inflammatory cells, giant cells, and formation of sinus tracts, and fibrosis. Involvement is typically in skin that contains apocrine sweat glands, but the inflammation and destruction of these glands seems to be incidental rather than a causative factor.

Attanoos et al. examined 118 pathologic hidradenitis specimens and found some degree of keratin plugging in all cases along with an active deep folliculitis. They concluded that plugging of the hair follicle itself led to apocrine inflammation, making the actual apocrine gland destruction of hidradenitis suppurativa a secondary process. These glands secrete a milky, odorless fluid that only becomes malodorous after it interacts with bacteria on the skin.

The apocrine glands secrete into the hair follicle as opposed to directly onto the skin like eccrine sweat glands. The function of apocrine secretion is unknown. Nevertheless, obstruction leads to secondary bacterial infection and rupture of the gland into the dermis and subcutaneous tissue, thus causing cellulitis, abscess, and draining sinuses. This process then leads to the characteristic “pit like” scars from chronic fibrosis of the destroyed glandular unit.

Over time, this disease can become not only disfiguring, but also debilitating. Microscopically, the pathognomonic serpentine epitheliazed sinus tracks with giant cells and granulomas are typically seen.

Clinical Presentation and Diagnosis

Patients with hidradenitis supprativa typically presents with pain, erythema, and swelling in the affected area. There is frequently a malodorous discharge from the affected skin. Physical examination may reveal a spectrum of indurated subcutaneous nodules, subcutaneous abscess, and/or draining skin sinuses. Sinuses may be simple or complex coalescing to form a network of subcutaneous cavities and tracts with extensive fibrosis.

The diagnostic is based on clinical findings, and diagnostic biopsy is seldom required; however, if the differential diagnosis includes perianal Crohn’s disease or cancer, biopsies should be obtained to establish a definitive diagnosis.

Differentiating hidradenitis supprativa from other inflammatory conditions of the perianal region can be difficult, and some of them may coexist. Cutaneous infections such as furuncles, carbuncles, lymphogranuloma venereum, erysipelas, epidermoid or dermoid cyst, and tuberculosis may present in a similar fashion.

It is important to distinguish hidradenitis from other fistulizing or sinus-forming processes of the perineum such as Crohn’s disease or cryptoglandular perianal abscess. Crohn’s disease typically affects the anus and rectum with fistulas arising from the dentate line or higher in the rectum. Fistula-in-ano or perianal abscesses that are cryptoglandular origin will arise from the dentate line and involve the sphincter complex.

In contrast, hidradenitis does not affect the rectum or involve the dentate line, because apocrine glands only exist in the lower two-thirds of the anal canal and do not penetrate into the sphincter complex. Thus, patients will not have sinus or fistula tracks to or from the rectum. If fistulas are present, then the surgeon should perform anoscopy to rule out the possibility of fistula-in-ano from a cryptoglandular source or consider Crohn’s disease as the aetiology.

Fistulas from hidradenitis should only connect areas of involved skin, and not penetrate the anal sphincters or involve the dentate line. Several case reports have been published describing the association of Crohn’s disease and hidradenitis, but no definitive link between the two conditions has ever been proven. Nonspecific granulomas (required for a pathologic specimens in both diseases and may be confused with one another.

In patients with longstanding history of hidradenitis and chronic nonhealing wounds, it is important to rule out malignancy. There have been several reports of squamous cell carcinoma arising in chronic hidradenitis wounds.

A retrospective review of a Swedish database of hospital discharge diagnoses from 1965 to 1997 revealed a 50% increased risk of developing any cancer in patients with hidradenitis supprativa over the general population, but significant increases were specifically found in nonmelanoma skin cancers, buccal cancer, and primary liver cancer.

Most patients with cancers had untreated disease for longer than 20 years. In fact, a recent review reports that the mean duration of hidradenitis suppurativa before diagnosis of malignancy is 25 years. One should at least keep a high index of suspicion for this entity in patients with long standing disease and extensive scarring in the affected areas.

Treatment

The clinical presentation of hidradenitis suppurativa encompasses a wide spectrum of severity. Furthermore, the persistent and recurring nature of the disease requires an individualized treatment plan. This may include nonsurgical and surgical techniques for acute and chronic disease.

It is imperative to educate the patient about the chronic relapsing nature of the disease, and to reassure the patient the disease is not contagious or due to poor hygiene.

  1. Nonsurgical Treatment

Antibiotic therapy is the cornerstone for nonsurgical treatment of hidradenitis. Patients with cellulitis and no definable abscess may be successfully treated with antibiotics for 1–2 weeks. Both topical (clindamycin) and systemic (tetracycline) antibiotics have been advocated, and antimicrobial spectrum must cover skin flora, particularly Staphylococcus species. No evidence exists supporting the use of prophylactic antibiotics beyond the initial treatment course, and it is unclear if the natural history or disease process is altered by such therapy.

Other medications have been used for the treatment of hidradenitis suppurativa, and include retinoids, antiandrogen therapy, immunomodulators and anti-inflammatory (etanercept, infliximab, adalimumab) drugs.

Most were evaluated through retrospective chart reviews and meaningful conclusions are difficult to draw; however, the potential side effects of these medications are significant and should be considered prior to initiation. Radiotherapy has been used in the past with modest success. These positive results are likely a direct result of hair follicle destruction, but wound healing problems are significant. Others have reported the use of photodynamic therapy cryosurgery, carbon dioxide laser therapy, and radiofrequency treatments with variable success.

  1. Surgical Treatment

The surgical management of hidradenitis suppurativa can be divided into two categories:

  1. surgery to control local infection; and
  2. surgery for curative intent.

Incision and drainage of abscess and sinus tracts are simple methods that control local infection; however, diseased skin remains and recurrence is highly likely. This is illustrated by a recent report that compared recurrence rates of hidradenitis suppurativa after incision and drainage/limited excision vs. wide excision of disease.

After a 3-month follow-up, the study found 100% recurrence after limited excision and 27% recurrence after wide excision of disease. These data suggest that the surgeon must excise the entire involved area, otherwise, the patient will be at risk for recurrence. For this reason, once local inflammation has been controlled with incision and drainage, the patient is offered further surgery for curative intent or initiated on nonsurgical treatment modalities for disease control.

Surgery for curative intent requires complete excision of diseased skin. Excision with primary closure may be performed in selected small wounds if it can be closed without lesions. This treatment modality results in decreased morbidity, decreased length of hospitalization and decreased postoperative disability.

Others have advocated wide excision and healing by secondary intention. All of the grossly involved apocrine bearing skin in the perianal area should be excised full-thickness into the uninvolved gluteal fat. Excision to involve a wide margin has proven to be beneficial. In addition, this method is simple, does not require fecal diversion, and depending on size excised, may be performed as an outpatient procedure. Patients with large areas of involvement may require staged excision. The extent of excision should remain outside the anal verge as long as there is no obvious involvement or history of involvement in the anal canal. If excision near the anal canal is necessary, it should be limited, or staged, in order to prevent anal stricture. Prolonged wound healing of 1 month or longer is a significant disadvantage of this method. These patients require daily wound care and consideration should be given to physical therapy to prevent contracture formation.

A male patient with significant perianal and inguinal hidradenitis suppurativa of a chronic nature
Figure X2. A male patient with significant perianal and inguinal hidradenitis suppurativa of a chronic nature. A | Preopertive photo of extent of disease. B | Planned surgical resection preserving skin at the anal verge. C | Wide excision of perianal and inguinal hidradenitis suppurativa. D | Split thickness skin graft (STSG) used for immediate reconstruction.

Recently, reports on the use of negative pressure dressings have appeared as a way to promote healing and shorten the time to wound closure. The purported benefits of these dressings include increased wound oxygen tension, decreased bacterial counts, better control of fluid produced by the wound, increased granulation tissue formation, and decreased shear forces.

Negative pressure dressings have been used successfully on open wounds and on skin grafts; however, consideration must be given to cost and technical difficulties inherent to dressings placed in the perianal area. These dressings require an air-tight seal at all times, which can be difficult to achieve near the anal verge and perineum.

Patients with chronic disease, extensive scarring, and sinus tracts rarely respond to conservative measures (Figure X2A). The gold standard of care remains wide excision of all hidradenitis involved skin bearing apocrine glands (Figure X2B and C). Reconstruction then can follow a number of paths — cutaneous flap closure, myocutaneous flap closure, immediate or delayed split-thickness skin grafting (Figure X2D), or excision, and simple healing by secondary intent.

Cutaneous or myocutaneous flaps are typically taken from the posterior thigh, gluteus muscle, or lumbosacral region. They are analogous to those used for pilonidal disease. Patients who might benefit from diversion are those who cannot take care of their wounds long term and those who suffer from both hidradenitis and Crohn’s disease, although this is rarely needed.

Hidradenitis suppurativa algorithm
Figure X3. Hidradenitis suppurativa algorithm


Summary

Hidradenitis suppurativa is a skin disease that typically affects young adults and is characterized by intermittent flares and periods of quiescence. The algorithm in Figure X3 outlines one suggested approach to treating patients with perianal hidradenitis suppurativa. Patients who present with acute disease and abscess should have incision and drainage, ideally in an office setting.

Antibiotics may be used for excessive cellulitis and to decrease acute inflammation. This process can be repeated, if necessary, and is usually performed for recurrent disease. It is important to rule out other causes of fistulous disease such as Crohn’s disease or perirectal abscess from a cryptogladular source.

For patients with chronic or recurring disease, definitive excision should be considered. This may include excision with primary closure for small areas of disease, healing by secondary intent for larger areas of disease, or reconstruction with split-thickness skin graft or flap closure for wide excision.

Flap procedures Opens in new window are typically reserved for patients with extensive scarring and tissue damage that involves large areas of perianal skin around the anus extending out to the buttocks. By the time a patient reaches the point where they desire surgery, they have usually suffered for many years with recurrent abscesses in the affected area.

See also:
    Adapted from: The ASCRS Textbook of Colon and Rectal Surgery: Second Edition.Edited By David E. Beck, Patricia L. Roberts, Theodore J. Saclarides, Anthony J. Senagore, Michael J. Stamos, Steven D. Wexner References as cited include:
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