Gluteal and Perianal Hidradenitis Suppurativa

Transcription

1 Gluteal and Perianal Hidradenitis Suppurativa Surgical Treatment by Wide Excision Sylvio F. Bocchini, M.D., Angelita Habr-Gama, M.D., Desidério R. Kiss, M.D., Antonio R. Imperiale, M.D., Sergio E. A. Araujo, M.D. From the Division of Colorectal Surgery, University of São Paulo, São Paulo, Brazil Address reprint requests to Dr. Habr-Gama: Manoel da Nóbrega, 1564, CEP São Paulo, Brazil. 944 PURPOSE: Hidradenitis suppurativa is a chronic inflammatory disease of the skin and subcutaneous tissue. Extensive gluteal and perianal disease represents a challenge presentation. The aim of this study was to present results of management of extensive hidradenitis suppurativa in gluteal, perineal, and inguinal areas. METHODS: From January 1980 to May 2000, 56 patients underwent treatment of hidradenitis suppurativa in gluteal, perineal, and inguinal areas through wide excision; 52 (93 percent) were male and 36 (64 percent) were white. Mean age was 40 years. We evaluated distribution of disease, associated conditions, use of diverting colostomy, management of operative wounds, time to complete healing, complications, and recurrence. RESULTS: Twenty-one (37.6 percent) and 17 (30.6 percent) patients had gluteal and perineal disease, respectively. Squamous-cell carcinoma and Crohn s disease were observed in one patient each. Wide surgical excision was performed in all. Healing by second intention was the choice in 32 (57.1 percent) patients, and 24 (42.9 percent) patients underwent delayed skin-grafting. Diverting colostomy was used in 23 (41 percent) patients. Mean time for complete healing in the nongrafted group was 10 (range, 7 17) weeks and in the skin graft group was 6 (range, 3 9) weeks. New resection was performed in five (8.9 percent) patients. Partial graft loss rate was 37.5 percent and recurrence was observed in only one (1.8 percent) patient. CONCLUSION: Significant morbidity derives from extensive gluteal and perineal hidradenitis suppurativa caused by the disease extension and large wounds that result from surgical treatment. Wide surgical excision is the treatment of choice and leads to cure. Skin-grafting and healing by second intention lead to effective wound healing. [Key words: Hidradenitis suppurativa; Skin graft; Apocrine glands; Abscess; Wound healing; Harmonic scalpel] Bocchini SF, Habr-Gama A, Kiss DR, Imperiale AR, Araujo SEA. Gluteal and perianal hidradenitis suppurativa: surgical treatment by wide excision. Dis Colon Rectum 2003;46: H idradenitis suppurativa (HS) is a chronic and recurrent inflammatory disease of the skin and subcutaneous cellular tissue, areas of the body where apocrine sweat glands are present. Regions most commonly affected are the axillae, perineum, gluteal, and inframammary area. Extensive gluteal and perianal HS is a relevant nosocomial problem, because it leads to intense suffering that results in physical, psychological, familiar, and professional disturbance. Surgical treatment is usually required, demanding radical extensive resection and may include the need of a temporary stoma. A multidisciplinary team is often required for appropriate care and long hospital stay and disability after discharge must be expected. When treatment is not performed in a radical way or when patients are not closely followed until definitive healing, recurrences are frequent. The current study was undertaken to present our experience with management of 56 consecutive patients with extensive HS in gluteal, perineal, and inguinal areas. PATIENTS AND METHODS Patients Patients with HS located in the perineal, inguinal, and gluteal areas, treated in the Colorectal Unit of the Gastroenterology Department at the University of São Paulo Medical School between January 1980 and May 2000 were reviewed. Extensive HS was defined as bilateral gluteal or perineal disease, because unilateral gluteal and perineal or unilateral gluteal and inguinal disease was present. Clinical data regarding gender, age, race, time of disease onset, location of the lesions, associated diseases, surgical treatment, time until complete healing, early and late operative complications, and recurrence rates were evaluated. Diagnosis of HS was based on clinical features and in all cases was confirmed by pathologic examination of the resected tissue. Serum levels of testosterone and androgens were measured in 21 (37.5 percent) patients. Methods Radical surgical excision under general or spinal anesthesia was performed in all patients. Patients

2 Vol. 46, No. 7 HIDRADENITIS SUPPURATIVA 945 Table 1. Distribution of Patients According to Location and Gender Location Male Female No. Patients Gluteal 19 (33.9) 2 (3.6) 21 (37.6) Perineal 15 (26.8) 2 (3.6) 17 (30.3) Gluteal and perineal 15 (26.8) 15 (26.8) Gluteal and inguinal 3 (5.3) 3 (5.3) Total 52 (92.8) 4 (7.2) 56 (100) Figures are given as. were operated in lithotomy, jackknife, or prone position. Operative technique included complete excision of the whole diseased skin and subcutaneous fatty tissue down to the muscular fascia under careful hemostasia. Excision was accomplished using cold blade, electrocautery, or harmonic scalpel. Lateral margins were taken up to healthy skin. Testicular tunics were also excised when needed because of extension of suppurative process and, as a rule, preservation of perianal skin was always attempted because there are no apocrine sweat glands in the close perianal area and in the anodermal region. When small satellite lesions were present, primary closure was always attempted. Secondary intention healing was used for larger lesions and delayed (after 2 or 3 weeks) skin grafting was preferentially used when extensive disease was present. Grafts were usually obtained from dorsal area of the thighs. Patients were seen weekly until complete wound healing, thereafter, monthly for three months and subsequently every four months thereafter until a year was completed. Recurrence was assessed by clinical signs and symptoms observed in the early postoperative period and later follow-up. RESULTS Fifty-six patients were enrolled in the study. Fiftytwo (93 percent) patients were male, and 36 (64 percent) were white. Mean age was 40 (range, 18 69) years. Duration of symptoms before first treatment varied between four months and 35 years, with an average of 7.2 years. Distribution of patients according to location and gender is presented in Table 1 and illustrated in Figure 1. Serum levels of testosterone, hydroxyprogesterone, and dehydroepiandrosterone were normal in the 21 patients undergoing such studies. Associated conditions were observed in 20 (35.7 percent) patients (Table 2). Squamous-cell carcinoma was observed in only one (1.8 percent) patient with a large gluteal Figure 1. Several degrees of gluteal and perianal hidradenitis suppurativa. Right inferior case (lithotomy position) with fistula formation. lesion. This 36-year-old male patient underwent multiple surgical interventions. Adjuvant radiotherapy and chemotherapy was also delivered with no success. He died after 18 months with disseminated disease. In 32 (57.1 percent) patients, the resultant wound was left to heal by second intention. In 24 (42.9 percent) patients, skin grafting was performed for wound closure within two or three weeks after excision when signs of sepsis were no longer observed (Fig. 2). Diverting colostomy was used in 23 (41 percent) patients because of extensive and complex lesions involving perianal margins. There were no complications related to the stoma construction or reversal, which was performed after complete wound healing. Mean time of follow-up was 12 months (range, 4 months to 6 years). Time for complete wound healing for patients treated by excision and second intention healing varied proportionally to the extent and degree of infection of the unroofed area, with a mean of ten weeks (range, 7 17 weeks). In the patients treated by excision and skin grafting, the time until complete

3 946 BOCCHINI ET AL Dis Colon Rectum, July 2003 healing was shorter, with a mean of six (range, 3 9) weeks. Partial loss of the skin graft occurred in nine (37.5 percent) patients treated with this technique. Two of these patients underwent new grafting with good results; the others were treated by surgical dressing and healing by second intention. Skin grafting provided a better cosmetic result, even when partial graft loss occurred, compared with healing by secondary intention. One patient received hyperbaric oxygen therapy because of severe infection of the grafted wound with recovery and complete second intention healing after two months of this treatment. One patient had anal stenosis, which was successfully treated by anal dilation (Table 3). Local recurrence was observed in one (1.8 percent) patient after 17 months. Management included reexcision and secondary wound healing. The patient declined further treatment. Five (8.9 percent) patients with persistence of the disease were managed by new local resection and secondary healing with good results. DISCUSSION HS is most likely caused by occlusion of the apocrine glandular duct by a keratinous plug, leading to ductal dilation and stasis in the glandular component. 1 As a consequence, secondary infection develops, with subsequent active folliculitis, followed by rupture of the gland and propagation of the infection to the contiguous glands and subcutaneous fatty tissue. Folliculitis seems to be the most important finding in pathologic specimens of HS and it is present in up to 67 percent of the lesions. 2 4 The cause of glandular obstruction remains unclear. Studies about the size and distribution of the apocrine glands; the use of deodorants, talc, chemical depilation, or blade depilation 5 ; and the influence of genetic factors and alterations of the immune state are not conclusive. 6 Mortimer et al. 7 observed elevated levels of androgens in patients with HS. Although considerable overlap with normal values was seen, antiandrogenic treatment was deemed effective. 8 In our study, serum levels of androgens were normal. There is no consensus about a relationship between HS and gender, race, and site of the lesions. 1 Current data are mostly based on small retrospective studies. Axillary location seems to be more frequent in women. 9,10 Inguinoperineal and gluteal locations are more prevalent in males. HS appears to be more common in young adults, 9,11 and onset is most commonly observed after puberty. In our study, 93 percent of the patients were male, in accord with the literature data. 12 Diagnosis of HS is primarily clinical. In the early stages of the disease, it presents with an abscess with or without cellulitis. The abscess may subside spontaneously after a few days or after simple incision and drainage, but usually recurs. The disease may progress to a chronic or recurrent form, with new contiguous or distant lesions, despite appropriate treatment. The final picture is the presence of multiple nodules, superficial sinuses, or fistulas surrounded by fibrosis and cellulitis in the perineum, inguinal region, gluteal area, and bases of the thighs, scrotum and vulva. Fistulous tracts are usually multiple and irregular. When the disease is limited to the perianal area, differential diagnosis of HS may be difficult because of other anal diseases such as cryptogenic anal fistulas, perianal Crohn s disease, pilonidal disease, or less Table 2. Distribution of Patients According to Associated Conditions Condition Perinal fistula 8 (14.3) Acne 6 (10.7) Diabetes mellitus 2 (3.6) Acne conglobata 1 (1.8) Squamous-cell carcinoma 1 (1.8) Gout 1 (1.8) Crohn s disease 1 (1.8) Figure 2. Gluteal and perianal extensive (left, superior) hidradenitis suppurativa. Treatment by wide surgical excision (right, superior) and delayed (15 days after excision) skin-grafting (left, inferior). Right inferior photograph was obtained nine months after grafting.

4 Vol. 46, No. 7 HIDRADENITIS SUPPURATIVA 947 common conditions (furunculosis, tuberculosis, or inguinal lymphatic granulomatosis). Association with small-bowel and large-bowel Crohn s disease was emphasized by Church et al. 13 in up to 39 percent of the cases. In our study, this association was observed in one (1.8 percent) patient alone. Knowledge of a patient s history and careful examination of axillae and the groin may reveal HS in these sites. Examination under anesthesia may be advisable when a patient presents with sepsis or cryptoglandular etiology is considered. Nevertheless, anal fistula may be an associated finding, as was the case in 14.5 percent of our patients. HS is frequently associated with simple acne or its more serious form, acne conglobata. Acne in its various forms was observed in 12.8 percent of our patients. Other conditions reported to be associated with HS are pilonidal disease, anal fistulas, spondylitis arthropathy, 14 arthritis, 15 diabetes mellitus, 16 and Cushing s syndrome. 17 Squamous-cell carcinoma arising in HS was observed in one (1.8 percent) patient. It is a rare but recognizable complication of HS Cancer risk is a well-known indication of surgical treatment, mainly in patients with longstanding lesions. Treatment has been accomplished through medical and surgical procedures. Conservative measures are indicated for small lesions or in an initial process of recurrence. Local treatments have included use of antiseptic solutions, topical use of zinc peroxide paste, or acetate of aluminum 21,22 and systemic antibiotic therapy with penicillin, tetracycline, chloramphenicol, and erythromycin. Ciprofloxacin may allow resolution in up to one-third of mild cases. 3,23 Although antibiotics are not curative, they may reduce odor, discharge, and pain. 24 Table 3. Complications Complication Partial graft loss 9 (16.1) Persistent disease 5 (8.9) Anal stenosis 1 (1.8) Local recurrence 1 (1.8) Radiotherapy was used in the past with apparently good results, 25 but there is a potential risk of radiodermatitis. Nonspecific immune modulation (staphage lysate), 26 anti-inflammatory inhibition of the glandular ducts cornification and sweat production (13 cis-retinoic acid (isotretinoin)), stimulation of T- cell suppressive activity (tolmetin sodium), 30 antiandrogens (cyproterone acetate and etinil-estradiol), 7,8 and, more recently, calcineurin inhibition through cyclosporine, 31,32 showed some efficacy for disease control. However, prospective studies are not yet available. Surgical approaches to the treatment of perianal, perineal, and gluteal HS include the lay open technique with curettage and healing by secondary intention 33,34 ; marsupialization; excision with primary closure; excision with healing by secondary intention or followed by skin-graft; and excision with CO 2 laser or harmonic scalpel. The choice of each technique is mainly determined by the clinical situation and surgeon preference. For early-onset disease limited to abscess, superficial sinuses, and fistulas, lay open, drain, and curettage may be the best alternative. 38,39 For deeper lesions still limited to a small area, when infection is mild or absent, excision with primary closure may be the best option. 34 Extensive disease causing significant symptoms requires wide excision and healing by either secondary intention or skin grafting (Table 4). Wide radical excision of the whole compromised area with enough depth until reaching health tissues has been preferable. 1,12,34,40 In our study, this method was used in all patients. During the operation, search for the presence of anal or rectal fistulas, and also for tracks to the ischiorectal spaces, is an important step. Suppuration may progress along the rectum and form ascending blind tracks through the levator ani. This finding was observed in 14.3 percent of our patients. After wide radical excision, wounds are treated preferably by delayed grafting. Despite considerable morbidity, mainly represented by lingering suppuration of the unroofed area and partial graft losses (37.5 Author, Year Table 4. Results of Surgical Treatment of Extensive Perianal Hidradenitis Suppurativa No. Patients Wide Excision, Diversion, Grafting, Recurrence, Wiltz et al., (72) 2 (5) 2 (5) 29 (67) Endo et al., (67) 0 5 (42) 2 (17)

5 948 BOCCHINI ET AL Dis Colon Rectum, July 2003 percent), all patients showed definitive healing and are disease-free. Recurrence rates of HS after surgical treatment may vary from 17 to 67 percent, 12,40 in a period of up to two-years follow-up, independent of the method used. Recurrences are almost always the result of an incomplete excision, although lesions at nonoperated sites may appear. In our study, local recurrence rate was 1.8 percent, proving the value of the radical wide excision. Temporary colostomy was used in patients with extensive and complex lesions (41 percent) involving anus and perianal margins to prevent wound infection and assist with local hygiene and dressing. CO 2 laser assisted excision has been proposed by some surgeons for the treatment of restricted and superficial lesions. According to these authors, it enables removal of affected tissues, better bleeding control and shorter hospital stay High cost and restricted availability represent the main disadvantages. Significant morbidity derives from extensive gluteal and perianal HS, mainly related to the extent of the suppurative process, need for radical surgical treatment that leads to a large wound, and, finally, the risk of recurrence. A careful evaluation of the distribution and extent of the lesions and presence of infection is required to determine optimal management. The main objectives of the surgery are to cure the disease and to reestablish function and cosmesis in the affected area. Our experience with radical wide excision followed by a selective approach of delayed skin grafting seems to represent an adequate management for extensive gluteal and perianal HS. This approach allowed us to achieve cure and wound healing in all patients. REFERENCES 1. Parks RW, Parks TG. Pathogenesis, clinical features and management of hidradenitis suppurativa. Ann R Coll Surg Engl 1997;79: Jemec GB, Hansen U. Histology of hidradenitis suppurativa. J Am Acad Dermatol 1996;34: Highet AS, Warren RE, Weekes AJ. Bacteriology and antibiotic treatment of perineal suppurative hidradenitis. Arch Dermatol 1988;124: Brook I, Frazier EH. Aerobic and anaerobic microbiology of axillary hidradenitis suppurativa. J Med Microbiol 1999;48: Morgan WP, Leicester G. The role of depilation and deodorants in hidradenitis suppurativa. Arch Dermatol 1982;118: Fitzsimmons JS, Guilbert PR, Fitzsimmons EM. Evidence of genetic factors in hidradenitis suppurativa. Br J Dermatol 1985;113: Mortimer OS, Dawber RP, Gales MA, Moore RA. A double-blind controlled cross-over trial of ciproterone acetate in females with hidradenitis suppurativa. Arch Dermatol 1986;115: Sawers RS, Randall VA, Ebling FS. Control of hidradenitis suppurativa in women using combined antiandrogen (ciproterone acetate) and oestrogen therapy. Br J Dermatol 1986;115: Golcman B, Tuma Junior P, Bonamichi GT, de Faria JC, Golcman R, Ferreira MC. Surgical treatment of hidradenitis suppurativa [in Portuguese]. Rev Hosp Clin Fac Med Univ S Paulo 1991;46: Knaysi GA Jr, Cosman B, Crikelair GF. Hidradenitis suppurativa. JAMA 1968;203: Barros DE, Resende MS, Macedo EJO. Tratamento cirúrgico da hidradenite supurativa perianal. Rev Bras Coloproctol 1988;8: Wiltz O, Schoetz DJ Jr, Murray JJ, Roberts PL, Coller JA, Weidenheimer MC. Perianal hidradenitis suppurativa. The Lahey Clinic experience. Dis Colon Rectum 1990; 33: Church JM, Fazio VW, Lavery IC, Oakley JR, Milsom JW. The differential diagnosis and comorbidity of hidradenitis suppurativa and perianal Crohn s disease. Int J Colorectal Dis 1993;8: Rosner IA, Richter DE, Huettner TL. Spondyloarthropaty associated with hidradenitis suppurativa and acne conglobata. Ann Intern Med 1982;97: Kenik J, Hurley J. Arthritis occurring during hidradenitis suppurativa. J Rheumatol 1985;112: Paletta C, Jurkiewicz MJ. Hidradenitis suppurativa. Clin Plast Surg 1987;14: Rogin JR, Pinkus H. Cushing s syndrome with hidradenitis suppurativa. Arch Dermatol Syph 1948;58: Black SB, Woods JE. Squamous cell carcinoma complicating hidradenitis suppurativa. J Surg Oncol 1982;19: Cricx B, Dontenwille MN, Grossin M. Carcinome epidermoide sur maladie de verneuil. Ann Dermatol Venereol 1983;110: Zachary LS, Robson MC, Rachmaninoff N. Squamous cell carcinoma occurring in hidradenitis suppurativa. Ann Plast Surg 1987;18: Brunsting HA. Hidradenitis suppurativa: abscess of the apocrine sweat glands. Arch Dermatol Syph 1939;39: Meleney FL, Johnson BA. Further laboratory and clinical experiences in the treatment of chronic, undermining, burrowing ulcers with zinc peroxide. Surgery 1937;1: Jemec GB, Faver M, Gutschik E, Wendelboe P. The bacteriology of hidradenitis suppurativa. Dermatology 1996;193:203 6.

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