Hidradenitis Suppurativa

Transcription

1 Hidradenitis Suppurativa Marc Singer, MD, and José R. Cintron, MD Hidradenitis suppurativa is a chronic inflammatory disorder of the apocrine sweat gland bearing skin. The progressive nature of the disease can make it extremely painful and severely debilitating. Traditionally, the etiology of the disorder was believed to be occlusion of the apocrine ducts. However, recent studies suggest that follicular occlusion is the initiating event. The most common sites of the disease are the axilla, perineum, external genitalia, and inguinal regions. Medical management may afford temporary relief of symptoms; however, most patients will eventually require surgical therapy. Incision and drainage or unroofing of sinuses may provide relief in select patients, but should be reserved for early and acute disease. Local excisions will provide adequate control of symptoms; however, greater than 50% recurrence can be anticipated. Wide excision with secondary granulation of perineal wounds will provide the most definitive therapy and can be accomplished safely. Perianal disease can more often be managed with local excision alone as recurrence rates of perianal disease are considerably lower than perineal disease Elsevier Inc. All rights reserved. Hidradenitis suppurativa is a chronic inflammatory disorder of the apocrine sweat gland bearing areas of the skin. The recurrent and progressive nature of this disorder can make it extremely painful and even incapacitating. Histologic examinations of affected skin suggest that the initiating event is follicular occlusion, with secondary occlusion and subsequent infection of the apocrine sweat glands. The sites most commonly affected include the axilla, perineum, genitalia, and inguinal regions. Medical management, including antibiotics and anti-inflammatory agents, can offer temporarily relief of symptoms; however, surgical excision of the effected apocrine bearing skin remains the only definitive treatment. From the University of Illinois at Chicago, Chicago, IL. Address reprint requests to José R. Cintron, MD, Associate Professor of Surgery, Division of Colorectal Surgery, University of Illinois at Chicago, 840 S. Wood Street, Room 518, M/C 958, Chicago, Illinois jcintron@uic.edu 2004 Elsevier Inc. All rights reserved /03/ $30.00/0 doi: /j.scrs ETIOLOGY In 1839 Velpeau first described hidradenitis suppurativa when he reported a patient with an inflammatory disorder of the skin at the axillary, mammary, and perianal regions. 1 French surgeon Verneuil later suggested such lesions represented an infection in the sweat glands. 2 In 1922, Schiefferdecker classified sweat glands as either apocrine or eccrine and made the association between hidradenitis and apocrine glands. 3 Finally in 1939, Brunsting detailed the histology of hidradenitis and suggested that obstruction of the apocrine glands is the initiating feature of the disease. 4 Apocrine glands are compound sweat glands located in the dermis and subcutaneous tissues. The glands contain a coiled secretory component that undergoes decapitation secretion into an associated pilosebaceous unit, which, in turn drains onto the skin. Under adrenergic control, apocrine sweat glands secrete a thick milky fluid in response to pain, anger, or sexual arousal. Initially, the apocrine secretions are odorless, but develop an odor after interacting with skin flora. There is no significant difference in the number, size, density, or distribution of apocrine glands in patients with hidradenitis compared with normal controls. 5 Since the 19th century, hidradenitis had been believed to be an apocrinitis caused by keratin plugging of the apocrine ducts. This belief was propagated by the work of Shelley and Cahn, 6 who applied atropine-impregnated tape to the axillary skin of normal volunteers. The tape caused keratinous plugging of apocrine ducts, and 3 of 12 volunteers developed inflammatory lesions closely resembling hidradenitis. They concluded that the tape occluded the apocrine ducts and reproduced hidradenitis. However, the experimental model produced lesions in only 25% of subjects, and the lesions were acute in nature, somehow different from the chronic, nonhealing wounds of true hidradenitis. Yu and Cook examined the resected axillary lesions of hidradenitis patients and found the most common histologic feature to be sinuses or cystic structures lined by stratified squamous epithelium in the dermis. 7 All of these structures contained keratin, and half contained hair shafts, suggesting that the sinuses were of follicular origin. Only one-third of cases displayed inflammation in the apocrine glands, and if present, the inflammation was also seen around the neighboring eccrine 186 Seminars in Colon & Rectal Surgery, Vol 14, No 4 (December), 2003: pp

2 HIDRADENITIS SUPPURATIVA 187 glands, follicles, and epithelium-lined cysts. None of the cases demonstrated gross dilation of the apocrine glands. Finally, the ruptured cysts had stratified squamous epithelium similar to the lining of hair follicles. The authors concluded that plugging and subsequent rupture of the hair follicles was the initiating event in hidradenitis. To further elucidate the etiology of hidradenitis, Attanoos and coworkers examined the histology of 118 resected specimens and identified a significant degree of follicular plugging, manifested as large cystic lesions, containing keratin and hair shafts. 8 Active inflammation of the sweat glands was uncommon, and when present, it was more frequent in eccrine glands. Inflammation found solely around apocrine glands was not present. In summary, the only consistent histologic feature of the disease was follicular plugging. Finally, Jemec and Hansen examined the resected specimens of 60 hidradenitis patients and again concluded that the majority of lesions were of follicular origin and only a minority were without a follicular component. 9 Apocrinitis was the dominant histologic feature in only 5% of all lesions. This collection of clinical and histologic evidence has established that the fundamental change of hidradenitis is the same as in acne vulgaris, that is, hyperkeratosis of the infundibulum, giving rise to comedo-like impactions. 10 Hidradenitis is not a primary disease of apocrine sweat glands as previously believed, in fact, it is an infundibulofolliculitis. 11 The plugged follicle ruptures, spilling corneocytes, bacteria, sebaceous matter, and hair into the connective tissues. 10 This stimulates an infiltrate of granulocytes and then mononuclear cells, which form foreign-body granulomas. This follicular plugging and subsequent inflammatory reaction cause secondary occlusion of the apocrine glands, leading to ductal dilation and stasis in the gland. Skin flora become trapped by the keratin plug and then multiply rapidly within the nutrientrich environment of the apocrine sweat. 12 The infected gland will eventually rupture into the surrounding dermis, resulting in a localized cellulitis and abscess formation. 13 Superinfection by skin flora can worsen the infection and contribute to the cellulitis, inflammation, tissue destruction, and skin damage. 14 The inflammation may become chronic, causing subcutaneous scarring, distortion of normal skin architecture, and characteristic pitlike scars. 15 Destroyed pilosebaceous units coalesce to form subcutaneous abscesses which discharge through multiple sites, resulting in extensive subcutaneous sinuses that rarely heal. A serpentine network of sinuses is considered pathognomonic for hidradenitis. 15 The stimulus for the initial follicular plugging remains unknown. A variety of predisposing factors have been considered. The disordered keratinization in hidradenitis may be related to androgen excess, due either to primary androgen secretion by the gonads or adrenals or to elevated levels of circulating free hormones. Apocrine sweat has also been shown to contain dihydroepiandrosterone sulfate which stimulates sebaceous activity. 16 Many authors have also suggested that obesity predisposes patients to hidradenitis, probably because of increased shearing forces on the affected skin. Although probably not the underlying cause, obesity certainly exacerbates existing disease, and weight loss remains a key principle of any management regiment. A review of patients treated at The Lahey Clinic documented that 70% of patients smoked, but a causal relationship could not be demonstrated. 17 The use of topical antiperspirants may prevent expulsion of apocrine secretions and contribute to the disease process. 18,19 In his review of 40 patients, Morgan found no significant differences in shaving, deodorant use, or chemical depilatories between hidradenitis patients and agematched controls. 20 Several investigators have proposed a genetic contribution or perhaps a familial variant of the disease Jemec et al found that 26% of hidradenitis patients had a positive family history compared to 2% of controls. 24 Von Der Werth et al also suggested an autosomal dominant inheritance for a familial form of hidradenitis. 25 There is no doubt that endocrine factors influence the pathogenesis of hidradenitis as evidenced by its development after the onset of puberty, and its rarity after age 40. Apparently the disease requires the presence of postpubertal sex hormones. Additionally, pregnancy, the later half of the menstrual cycle, and the use of oral contraceptives are known to activate hidradenitis, perhaps because androgens increase keratin production The disease can also be found in patients with overt endocrine disorders such as diabetes mellitus, Cushing s disease, and acromegaly. 32,33 Despite numerous publications relating hidradenitis to a

3 188 SINGER AND CINTRON variety of genetic, endocrine, and immunologic abnormalities, there remains no clear association with any of these. INCIDENCE The true incidence of hidradenitis is difficult to assess as the disease is frequently misdiagnosed or remains untreated for many years. Conflicting evidence exists as to whether the disorder is more common in men or women. 24,27,29,34-36 Fitzimmons and coworkers have estimated an incidence of 1 per 300 adults. 23 The incidence has been suggested to be as high as 4% in women. 28 Jemec and coworkers found that the point prevalence in a younger adult population in Denmark was as high as 4.1%. However, this was acquired by a patient survey so responses may have included other diagnoses. 24 One study in which 1000 dermatology patients were examined found a point prevalence of 1 per There is some evidence that the disease may be more common in African-Americans than Caucasians. 34 Axillary disease is more common in women, 19,38 while perianal disease is more common in men. 17,27,35 The disease almost always presents after puberty, although nine cases of prepubescent hidradenitis have been reported in the absence of endocrine abnormalities or androgen excess. 39,40 The peak onset is between ages 15 and 30, which coincides with the postpubertal increase in androgen levels. Thornton and Abcarian reported that 78% of patients were less than 31 years old. 34 The extreme rarity of hidradenitis in prepubertal patients or patients more than 40 years old emphasizes the hormonal influence of the disease. CLINICAL COURSE Hidradenitis is known to affect all anatomic regions containing apocrine glands; however, the axilla and inguinal perineal regions are the most commonly affected. 41 Other apocrine-bearing sites which can become effected include the areola, submammary region, periumbilical region, scalp, face, external auditory meatus, nape of the neck, and shoulders. Diagnosis of early lesions can be difficult, as the appearance may resemble cutaneous infections such as acne, cellulitis, epidermoid cysts, dermoid cysts, furuncles, carbuncles, lymphogranuloma venereum, erysipelas, nocardial infection, actinomycoses, tularemia, chronic pyogangrenosum, or tuberculosis. The initial presentation typically includes pain, induration, and deepseated nodules. The induration will then suppurate and develop into an abscess surrounded by cellulitis. Frequently, the abscesses spontaneously drain small volumes of malodorous discharge. The wounds may heal, but more often they do not heal completely and recur in time. The repeated infections result in larger abscess cavities and the formation of chronically draining sinuses and interconnected cutaneous fistulae all within a hard fibrotic wound. Subcutaneous tunneling involving extensive areas of skin is common. The hidradenitis does not usually penetrate beyond the deep fascial barrier, even in advanced disease. The severity of disease is highly variable. Some patients exhibit only mild disease in limited areas, while others will develop extensive involvement of multiple sites. Remission and relapse is typical. Lesions may resolve for years, and later recur at the same site or elsewhere. 15,16,27 The large draining wounds can become quite debilitating. Painful wounds, particularly at the axilla and perineum, can restrict movement and normal physical activity. The foul-smelling discharge can become an embarrassment, and the disease can become socially devastating in severe cases. The severe morbidity of hidradenitis has been documented objectively using the Dermatology Life Quality Index. 42 The follicular occlusion theory of hidradenitis suppurativa bears resemblance to a series of other conditions that collectively have become known as the follicular occlusion triad. This triad consists of acne conglobata, dissecting cellulitis of the scalp (Perifolliculitis capitis) and hidradenitis suppurativa. 43 Acne conglobata is a severe form of acne involving the chest, back, and buttocks, characterized by comedones and multiple areas of small purulent nodules. Perifolliculitis capitis is a condition of inflammation and pustular nodules on the scalp. The chronic inflammation and scarring leads to focal alopecia. A fourth component, pilonidal sinus, has been added to form the follicular occlusion tetrad. The continuous drainage and chronic inflammatory state of hidradenitis can lead to physiologic changes such as hypoproteinemia or iron-deficient anemia. 44 Other complications include intestinal keratitis, osteomyelitis, fistulae to pelvic organs, amyloidosis, and even death. 27,45 Several reports of associated arthropathies suggest an immune mediated component of hidradenitis

4 HIDRADENITIS SUPPURATIVA 189 Squamous cell carcinoma is an infrequent, but well described complication of hidradenitis. There are less than 30 reported cases in the literature. One author found an incidence of 3.2% in 125 cases of hidradenitis. 51 Perez-Diaz et al reviewed all published cases of squamous cell carcinoma arising within hidradenitis and found the average age at diagnosis was 47 years, with a male predominance. 52 The carcinoma occurred most frequently in the perineal, gluteal, and perianal regions, but almost never in the axilla. The average duration of hidradenitis at the time of diagnosis of the squamous cell carcinoma was 20 years. Treatment ranged from wide local excision to abdominoperineal resection with chemoradiation. In a series of patients hospitalized for hidradenitis, Lapins et al reported that the risk for cancer of any site was increased by 50% compared with matched controls in Sweden. Additionally, the risk of squamous cell carcinoma of the skin was increased 4.6-fold. 53 As this series included only hospitalized patients, it may represent a subset of patients with only the most severe disease. However, it clearly demonstrates an increased risk of cancer related to hidradenitis. It is the surgeon s responsibility to request that all histologic specimens be specifically evaluated for squamous cell carcinoma. Perianal hidradenitis will present with pain, swelling, purulent discharge, pruritus, or bleeding. The multiple sinus tracts and abscesses embedded within the dense fibrous tissue of the perineum and buttocks can cause severe pain. Perianal disease can be particularly difficult to diagnose due to the high incidence of other perianal pathology that mimics hidradenitis. Perianal lesions must be differentiated from other more common sources of perianal disease such as perianal abscess or fistula in ano of cryptoglandular origin. Patients will often give a history of previous operations for misdiagnosed fistula in ano. Fistulae-in-ano arise from the dentate line and involve the intersphincteric plane; however, tracts from hidradenitis will not penetrate the internal sphincter. Only the distal two-thirds of the anal canal contains apocrine glands, so the dentate area will be spared in hidradenitis. 54 Endo et al reported a series of 10 patients with perianal hidradenitis in which seven patients were found to have coexisting fistulae in ano. 55 This is an unusually high incidence of concurrent fistulae; however, it does emphasize the need for a thorough perianal examination. Failure to diagnose and appropriately treat the fistula in ano would result in ongoing perineal sepsis even after adequate treatment of the hidradenitis. Hidradenitis is often diagnosed as a pilonidal sinus, especially if the sinuses track toward the postsacral region. The symptoms of early hidradenitis and an infected pilonidal sinus may be identical. In addition, hidradenitis must be differentiated from perianal Crohn s disease. These two can be indistinguishable and the presence of one certainly does not rule out the other. Several reports of simultaneous Crohn s disease and hidradenitis have been published, and some authors believe that the two may in fact be related. 54,56,57 If perianal Crohn s disease is suspected, then cutaneous biopsy and colonoscopy with biopsy are indicated. Perianal Crohn s without colorectal involvement is rare, and this diagnosis should be made with caution. Hidradenitis misdiagnosed as Crohn s disease may result in delayed surgical therapy. More importantly, wide surgical excision of perianal Crohn s disease misdiagnosed as hidradenitis may result in an extensive nonhealing wound, which could be devastating to the patient. Church et al suggested that the perianal swelling and inflammation associated with Crohn s disease may precipitate the development of perianal hidradenitis in patients prone to it, although this offers no explanation of the presence of hidradenitis in other sites. 58 The treatment of patients with hidradenitis and Crohn s disease is difficult and the clinical course is considerably more severe than other patients. In a series of such patients treated at the Cleveland Clinic, 70% required proctectomy and over 90% required fecal diversion. 58 MICROBIOLOGY The microbiological flora of hidradenitis is inconsistent and difficult to characterize. Thornton and Abcarian found negative cultures in half of patients treated for perianal and perineal disease. 34 In positive cultures, Staphylococcus epidermidis, Escherichia coli, klebsiella, proteus, alpha streptococcus, anaerobes, and diptheroids have been isolated. Highet et al suggested Streptococcus milleri, part of gastrointestinal and vaginal flora, as the pathogenic organism in perineal hidradenitis. 13 They isolated this organism from 21 of 32 patients with perineal hidradenitis and found disease activity to significantly correlate with its presence. Others have suggested that Chlamydia trachomatis or

5 190 SINGER AND CINTRON Bilophila wadswothia may have a pathogenic role, but further confirmation is needed. 59,60 Notably absent from most cultures is Propionibacterium acnes, the pathologic organism in acne. TREATMENT When planning a treatment regimen for any patient with perineal or perianal hidradenitis, a thorough history and physical are essential. Other sites of hidradenitis must be discovered and treatment prioritized. If disease of the axilla, for example, is more severe and debilitating, then that site should be addressed first. A complete history of other perianal pathology must be elicited, including sexually transmitted diseases, history of incontinence, or prior operative procedures for any indication. The surgeon must investigate the involvement of organs other than the skin, including intestinal or urinary fistulae. MEDICAL THERAPY Initially, hidradenitis may be treated with conservative measures for immediate relief of pain and other symptoms. Sitz baths, whirlpool therapy, and topical cleansing agents can be used to maintain perineal hygiene, remove drainage, and reduce bacterial load. 61 Loose clothing should be worn, and obese patients should begin dieting. None of these measures will provide long-term relief or cure; however, they may be helpful as more definitive therapy is planned. Antibiotic therapy can be used as an adjunct to surgical therapy, but generally cannot be used to control the disease. Topical clindamycin is the only antibiotic that has been shown to be beneficial in a prospective, randomized, controlled trial. 61 Systemic tetracycline has been shown to be at least as beneficial as topical clindamycin. 62 If antibiotic therapy is used for perineal disease, then broad spectrum antibiotics should be used initially, and then therapy tailored to culture results. There is no evidence that chronic antibiotic therapy can control the disease. 41,54 Although the exact mechanism of hormonal influence on hidradenitis is unclear, some physicians have attempted to use hormonal therapy as treatment. Steroids have successfully been used to reduce the local inflammation in the wound, but also to suppress the hypothalamic-pituitary axis. 63 Inhibition of that axis can also be accomplished with the use of leuprolide, a synthetic gonadotropinreleasing hormone, which has been demonstrated to improve hidradenitis in some patients. 64 Also, therapy with cyproterone acetate, an antiandrogen, has improved symptoms in European trials, although the drug is not available in the United States. 65,66 Isotretinoin, a derivative of vitamin A, has been used for nearly 20 years to successfully treat acne by decreasing epithelial differentiation and sebum secretion; however, results for hidradenitis have been mixed. 67,68 There are no established dosing guidelines for its use, and it carries many known complications including altered serum lipid profiles, hepatic dysfunction, and pseudotumor cerebri. In addition, it is a potent teratogen, making strict use of contraception in women mandatory. Recent studies have also shown that etretinate and acitretin can be somewhat effective in treating hidradenitis Cyclosporine has been used successfully to manage hidradenitis, but further investigation is necessary, especially in light of the serious side effects of cyclosporine therapy. 72 The association between hidradenitis and Crohn s disease has been suggested by several authors 56-58,63,73-76 ; however, the exact mechanism of this association remains unclear. Martinez et al 77 reported the successful treatment of hidradenitis with infliximab (anti-tnf antibody) in a patient that also suffered perianal Crohn s disease. This encouraging result suggests that immunomodulators, such as infliximab, may represent a potentially useful class of drugs to treat hidradenitis. INCISION AND DRAINAGE When he described hidradenitis suppurativa in 1854, Verneuil recommended treatment by incision and drainage of all fluctuant areas. 2 This method of treatment remains useful in the management of acute symptoms; however, even the most meticulous drainage of the perineal area will result in a high incidence of recurrence. 44,78,79 Local incisions are best used to relieve undrained pus, as a bridge to more definitive therapy. UNROOFING Unroofing of sinus tracts can be helpful, despite the fact that recurrence rates will be suboptimal. 78,79 This technique may be valuable if perineal disease is extensive, with extension onto the inguinal regions and genitalia. Under anesthesia, all

6 HIDRADENITIS SUPPURATIVA 191 sinus and fistula tracts should be thoroughly explored and probed. Each tract should be unroofed; the track should be curetted, and the epithelialized floor left intact. The wounds may be marsupialized. Postoperative sitz baths, whirlpool therapy, and aggressive wound care are essential. Subsequent unroofing procedures may be required to adequately treat all tracks. The decision to treat perineal disease in this manner should be the decision of the patient after discussion with the surgeon, caretaker, family, etc. The patient must understand that he or she will be accepting a higher rate of recurrence in exchange for a less extensive surgical debridement, and the possibility of an occult squamous cell carcinoma. The best role for unroofing of sinuses is to relieve symptoms in early and acute disease, while allowing time to plan definitive therapy. LOCAL EXCISION Local excisions are frequently performed to control local infection without the morbidity of an extensive excision. As a general rule, local excisions will result in a high rate of recurrence; however, for unclear reasons perianal (5%) disease offers a better result when compared with perineal lesions (37-74%). 27,41,80-82 A well-performed local excision in combination with unroofing of fistulae and adequate wound care can sometimes offer acceptable results. 15 Again, patients must weigh the risk of recurrence against the morbidity of a larger excision. Factors associated with high rates of recurrence after local excision include inadequate excision, obesity, excessive skin maceration, and chronic skin sepsis. 41 WIDE EXCISION Wide excision of all involved apocrine gland bearing skin is the most successful treatment of hidradenitis. Once the disease process has become chronic and extensive, many authors would agree that wide excision offers the most satisfactory results. 15,34,55,80,81,83-88 Preoperative preparation is most important with wide excision, as the patient will suffer significant short-term morbidity. The patient should fully understand the extent of the planned operation and be prepared to attend to the wounds postoperatively. In addition, the patient should be psychologically prepared for the burden of caring for the anticipated wounds. If necessary, the patients nutritional status should be optimized. The patient should receive either regional or general anesthesia and is best positioned in either the prone jackknife or the supine lithotomy position, depending on the extent of the debridement. The lithotomy position provides good exposure for a combined perineal and inguinal resection. The entire area of affected skin should be resected down to normal-appearing deep fascia, or at least 5 mm of subcutaneous fat, to ensure that all apocrine glands have been removed. 12 The resection should also include a 1 to 2 cm lateral margin of normal appearing skin. Some authors recommend that when excising perineal hidradenitis, a bridge of skin should be maintained between the anus and genitalia. 12 Also, in patients with extensive perineal disease, staged excision, by quadrant may maintain more normal anatomy after healing. Some authors advocate the mapping of apocrine gland bearing skin with the iodine-starch test to facilitate a complete excision. This technique involves the blocking of eccrine secretion with intravenous atropine, followed by the administration of intravenous oxytocin to stimulate the myoepithelial cells surrounding the apocrine glands. Iodine is then topically applied to the skin, followed by starch powder in castor oil. The apocrine sweating can be identified by the appearance of black dots which mark the areas where the sweat contacted the iodine-starch mixture. 5 After surgical resection, the wound can be managed in several ways: primary closure, coverage with split thickness skin grafting, coverage with flaps, or closure by secondary granulation. Rompel and Petres demonstrated in patients with hidradenitis at various sites, that recurrence is related to severity of disease and adequacy of resection, not to method of reconstruction. 36 The choice of reconstruction is based on postoperative wound management and complications, not recurrence of disease. Recurrence is likely if the excision is inadequate or if there is an unusually wide distribution of apocrine glands. If primary closure is being considered, the extent of excision must never be compromised, as disease will surely recur. Closure can be attempted if possible, especially if the defect is small and can be closed without flaps or skin grafting. Closure of extensive perineal defects should not be attempted. Skin grafting of perineal wounds may be performed immediately

7 192 SINGER AND CINTRON after excision or in a delayed fashion, after the wound has started to granulate. 88 Skin grafting carries several drawbacks including prolonged operative time, donor site morbidity, need for immobilization, graft loss, and frequent dressing changes. The skin grafts can be either stapled or sutured to the periphery of the wound and should be meshed to allow drainage. The patient can begin limited ambulation after several days. Healing of the graft should be complete within 2 weeks postoperative. Immediate grafting in the perineum is not recommended as the bacterial load leads to prohibitive rates of graft failure. 15,83 Harrison reported a 45% failure ( 50% take) rate of skin grafts. 41 Skin grafting is not recommended in the anal canal as contraction of the graft may lead to anal stenosis. Furthermore, graft failure and synergistic infection may lead to aggressive infections, Fournier s gangrene, or deep scarring and subsequent sphincter damage. Perioperative antibiotics should be used if wounds are closed or grafted. Flap coverage can be performed to cover perineal excisions. Possible donor sites include the posterior thigh, the gluteus maximus, and the lumbosacral area of the back. Flap coverage of wounds does allow closure of the defect with early return to normal activity. However, the wounds are generally extensive and require multiple large flaps, which increases operative time, blood loss, postoperative pain, and the possibility of infection or recurrence of hidradenitis beneath the flap. Perineal flaps should generally not be used unless vital structures, such as nerves, vessels, or bone, require coverage after debridement. 87 Many investigators argue that perineal wounds are best left open to granulate. Allowing the open wound to granulate will afford the patient earlier ambulation, avoid donor site pain and scarring, and earlier hospital discharge. If the wound is properly managed, then secondary granulation will give excellent results in most cases of even very large perineal excisions. 17,34,88,89 The contractures will be minor; the scars will remain soft and pliable, and the physical restrictions on the patient are minimal. If the wound is left open, then antibiotics are not indicated. 34 Most wounds will heal by secondary intention with very little deformity within 3 to 8 weeks postoperative. 87 If the wound is allowed to granulate for several months, but it will not completely close, then delayed skin grafting should be considered. In their series of 104 patients with perianal and perineal hidradenitis, Thornton and Abcarian performed wide excision of the involved area down to the normal fascia. 34 Skin grafts and colostomies were not routinely used. Patients averaged 7.2 days in the hospital and 80% of patients were home by postoperative day 10. Average healing times ranged from 3.5 to 7 weeks. Recurrence occurred in four (4%) patients during the 5-year study period. Silverberg et al reviewed a series of patients that underwent perineal resections averaging 300 cm 2 and were allowed to heal by granulation. 90 The operative time averaged less than 1 hour. Patients were encouraged to walk as tolerated, and analgesic requirements were minimal by postoperative day 3 or 4. Patients were discharged from the hospital in 6 days on average. After 1-year follow-up, all patients had uncomplicated, well-healed wounds, with unrestrictive scars. In a series of 82 patients treated by wide excision, recurrence rates for perianal disease was zero, 3% for axillary, and 37% for inguinoperineal disease. 41 This highlights the fact the inguinoperineal region contains a more diffuse distribution of apocrine glands, making complete excision difficult. It also emphasizes good results for perianal disease. It should be noted that fully 25% of recurrences occurred at sites not previously involved with hidradenitis. In their series of selected patients with extensive and severe perineal disease, Bocchini and coworkers 88 reported only a 1.8% recurrence rate after wide excision. This data confirm that adequate excision of all effected skin down to the muscular fascia is the mainstay of therapy. The method of wound management does not influence recurrence. Routine colostomy is unnecessary in most cases. Even large areas of perineal debridement can be kept clean with aggressive wound care. If the surgeon believes that proper wound care will not be possible, then fecal diversion may be considered, for example, if the patient cannot ambulate to take frequent sitz baths or if morbid obesity prevents wound care. The other group of patients which may require fecal diversion are patients suffering from simultaneous Crohn s disease as well as hidradenitis. CONCLUSIONS The management of perineal hidradenitis must be individualized according to the location and extent of disease. Short-term antibiotics and wound

8 HIDRADENITIS SUPPURATIVA 193 care may be helpful as a temporizing measure while planning more definitive therapy. Hormonal therapy and vitamin A derivatives can be used for early disease. Incision and drainage or unroofing of sinuses may provide relief in select patients, but should generally be reserved for relief of symptoms in early and acute disease. Local excisions will provide adequate control of symptoms; however, greater than 50% recurrence can be anticipated. Wide excision will provide definitive therapy and can be accomplished safely. Advancement flaps or skin grafts can be used to cover larger wounds; however, secondary granulation will provide good results and high patient satisfaction, without the complications associated with flaps or grafts. Perianal disease can more often be managed with local excision alone. Recurrence rates of perianal disease are considerable lower than perineal disease. Primary closure can be attempted if the defect is small. Intraanal grafts should be avoided so as to avoid rejection, infection, scarring, and anal stenosis. 1. Velpeau A: in Aissele, Dictionnaire de Medicine, on Repertoire General des Sciences Medicales sons le Rapport Theorique et Pratique Behcet Jeune Z (ed) 2:91, Verneuil A: Etudes sur les tumeurs de la peau et quelques maladies des glandes sudoripares. Arch Gen Med 94: , Schiefferdecker B: in Die Hautdrusen des Menschen, und der Saugetiere, ihre Histologische und rassenanatomische Bedeutung Sowie die Muscularis Sexualis. Stuttgart: E Schweizerbart, Brunsting HA: Hidradenitis suppurativa: abscess of the apocrine sweat glands. Arch Derm Syphil 39: , Morgan WP, Hughes LE: The distribution, size and density of the apocrine glands in hidradenitis suppurativa. Br J Surg 66: , Shelley WB, Cahn MM: The pathogenesis of hidradenitis suppurativa in man. Arch Derm Syphil 72: , Yu CCW, Cook MG: Hidradenitis suppurativa: a disease of follicular epithelium, rather than apocrine glands. Br J Dermatol 122: , Attanoos RL, Appleton MAC, Douglas-Jones AG: The pathogenesis of hidradenitis suppurativa: a closer look at apocrine and apoeccrine glands. Br J Dermatol 133: , Jemec GBE, Hansen U: Histology of hidradenitis suppurativa. J Am Acad Dermatol 34: , Jansen T, Plewig G: Acne Inversa. Int J Dermatol 37: , Boer J, Weltevreden EF: Hidradenitis suppurativa. A clinicopathological study of early lesions. Br J Dermatol 135: , Parks RW, Parks TG: Pathogenesis, clinical features and management of hidradenitis suppurativa. An R Coll Surg Engl 79:83-39, Highet AS, Warren RE, Weekes AJ: Bacteriology and antibiotic treatment of perineal suppurativa hidradenitis. Arch Dermatol 124: , Ebling FJ: Apocrine glands in health and disorder. Int J Dermatol 28: , Rubin RG, Chinn BT: Perianal hidradenitis suppurativa. Surg Clin North Am 74(6): , Keighley MR, Wiilliams MS: Surgery of the Anus, Rectum and Colon (2 nd ed). London, Saunders, 1999, pp REFERENCES 17. Wiltz O, Schoetz DJ, Murray JJ, et al: Perianal hidradenitis suppurativa. The Lahey Clinic experience. Dis Colon Rectum 33: , Broadwater JR, Bryant RL, Petrino RA, et al: Advanced hidradenitis suppurativa: review of surgical treatment in 23 patients. Am J Surg 144: , Rogers IW, Ryan RF: Surgical treatment of hidradenitis suppurativa. J La State Med Soc 10:21-24, Morgan WP: The role of depilation and deodorants in hidradenitis suppurativa. Arch Dermatol 118: , Fitzsimmons JS, Fitzsimmons EM, Gilbert G: Familial hidradenitis suppurativa: evidence in favour of single gnee transmissions. J Med Genet 21: , Fitzsimmons JS, Gilbert G: A family study of hidradenitis suppurativa. J Med Genet 22: , Fitzimmons JS, Guilbert PR, Fitzimmons EM: Evidence of genetic factors in hidradenitis suppurativa. Br J Dermatol 113:1-8, Jemec GBE, Heidenheim M, Nielsen NH: The prevalence of hidradenitis suppurativa and its potential precursor lesions. J Am Acad Dermatol 35: , Von Der Werth JM, Willimas HC, Raeburn JA: The clinical genetics of hidradenitis suppurativa revisited. Br J Dermatol 142: , Stellon AJ, Wakeling M: Hidradenitis suppurativa associated with use of oral contraceptives. BMJ 298:28-29, Banerjee AK: Surgical treatment of hidradenitis suppurativa. Br J Surg 79: , Jemec GBE: The symptomatology of hidradenitis suppurativa in women. Br J Dermatol 119: , Harrison BJ, Kumar S, Read GF, et al: Hidradenitis suppurativa: evidence for an endocrine abnormality. Br J Surg 72: , Ebling FJG: Hidradenitis suppurativa: an androgen-dependent disorder. Br J Dermatol 115: , Lewis F, Messenger AG, Wales JKH: Hidradenitis suppurativa as a presenting feature of premature adrenarche. Br J Dermatol 129: , Chalmers RJG, Ea RD, Beck MH, et al: Acne vulgaris and hidradenitis suppurativa as presenting features of acromegaly. BMJ 287: , Curtis AC: Cushing s syndrome and hidradenitis suppurativa. Arch Dermatol Syph 62: , 1950

9 194 SINGER AND CINTRON 34. Thornton JP, Abcarian H: Surgical treatment of Perianal and perineal hidradenitis suppurativa. Dis Colon Rectum 21: , Brown SC, Kazzazi N, Lord PH: Surgical treatment of perineal hidradenitis suppurativa with special reference to recognition of the perianal form. Br J Surg 73: , Rompel R, Petres J: Long-term results of wide surgical excision in 106 patients with hidradenitis suppurativa. Dermatol Surg 26: , Lookingbill DP: Yield from a complete skin examination. J Am Acad Dermatol 18:31-37, Cornbleet T: Pregnancy and apocrine gland disease: hidradenitis and Fox-Fordyce disease. AMA Arch Dermatol Syph 65:12-19, Mengesha YM, Holcombe TC, Hansen RC: Prepubertal hidradenitis suppurativa: two case reports and review of the literature. Pediatr Dermatol 16(4): , Palmer RA, Keefe M: Early-onset hidradenitis suppurativa. Clin Exp Dermatol 26:501-3, Harrison BH, Kumar S, Read GF, et al: Recurrence after surgical treatment of hidradenitis suppurativa. 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10 HIDRADENITIS SUPPURATIVA Tsianos EV, Dalekos GN, Tzermias C, et al: Hidradenitis suppurativa in Crohn s disease. A further support to this association. J Clin Gastroenterol 20:151-3, Roy MK, Appleton MA, Delicata RJ, et al: Probably association between hidradenitis suppurativa and Crohn s disease: significance of epithelioid granulomas. Br J Surg 84: , Martinez F, Nos P, Benlloch S, Ponce J: Hidradenitis suppurativa and Crohn s disease: response to treatment with infliximab. Inflamm Bowel Dis 7: , Jemec GB: Effect of localized surgical excisions in hidradenitis suppurativa. J Am Acad Dermatol 18: , Paletta C, Jurkiewicz MJ: Hidradenitis suppurativa. Clin Plast Surg 14: , Anderson MJ, Docketry MD: Perineal hidradenitis suppurativa. Dis Colon Rectum 1:23-31, Watson JD: Hidradenitis suppurativa a clinical review. Br J Plast Surg 38: , Williams ST, Busby RC, De Muth RJ, et al: Perineal hidradenitis suppurativa: presentation of two unusual complications and a review. Ann Plast Surg 26: , Knaysi GA, Socman F, Crickelair GF: Hidradenitis suppurativa. JAMA 203:19, Shaughnessey DM, Greminger RR, Margolis IB, et al: Hidradenitis suppurativa: a plea for early operative treatment. JAMA 222: , Conway H, Stark RB, Climos S, et al: The surgical treatment of chronic hidradenitis suppurativa. Surg Gynecol Obstet 95: , Ritz JP, Runkel N, Haier J, Buhr HJ: Extent of surgery and recurrence rate of hidradenitis suppurativa. Int J Colorect Dis 13: , Weinzweig N: Hidradenitis Suppurativa, in Cohen M (ed): Mastery of Plastic and Reconstructive Surgery. New York, Little, Brown and Co, 1994, pp Bocchini SF, Habr-Gama A, Kiss DR, Imperiale AR, Aruajo SEA: Gluteal and perianal hidradenitis suppurativa: surgical treatment by wide excision. Dis Colon Rectum 46: , Mason JK: Surgical treatment for hidradenitis suppurativa. Surg Clin North Am 49: , Silverberg B, Smoot CE, Landa SJ, et al: Hidradenitis suppurativa: patient satisfaction with wound healing by secondary intention. Plast Reconstruc Surg 79: , 1987