Review Br. J . Surg. 1992, Vol. 79, September, 863-866
A. K. Banerjee Department of Surgery, Sandwell District General Hospital, Lyndon, West Bromwich, Birmingham B714HJ, UK Correspondence to: Mr A. K. Banerjee
Surgical treatment of hid radenitis su ppurat iva The management of hidradenitis suppurativa is frequently unsatisfactory. No method satisfies all requirements f o r the ideal treatment quick healing, no hospital admission, minimal patient inconvenience and low recurrence - but greater awareness of the strengths and weaknesses of existing methods should lead to improved management. Good reports of the relative cure rates of the diflerent surgical options are scarce and controlled trials non-existent, but existing evidence is reviewed critically and summarized. The operation of choice in most instances is wide local excision and healing .by secondary intention.
The ideal treatment of hidradenitis suppurativa ( H S ) should provide a high likelihood of cure with a low recurrence rate, should avoid hospital admission and general anaesthesia, and should involve minimal inconvenience and time off work for the patient. The disease shows a wide spectrum of severity; many cases remain mild and never progress beyond one site, and some undergo spontaneous remission. However, some untreated patients can be expected to progress relentlessly until total involvement of individual and additional apocrine areas is complete’. Properly diagnosed and adequately treated, early stages of the disease can often be controlled with medical measures. However, in established HS there is no evidence that treatment other than surgery has any effect on the natural history of the condition. As the disease becomes more chronic, obtaining complete resolution is increasingly difficult and in advanced cases may be impossible’. HS is a chronic, suppurative and cicatricial disease of apocrine gland-bearing skin areas, principally the axillae, anogenital skin and, rarely, the breasts. The disease was first documented in 1839 by Velpeau3, who reported a peculiar localization of cutaneous abscesses to the axillary, mammary and perianal skin; both Lane4 in 1933 and Brunsting’ in 1939 described the clinical features of the disease more fully and its frequent association with acne. Shelley and Cahn demonstrated an experimental model of the disease6.
Incidence HS occurs in both sexes7, although women develop the axillary form more frequently, while men show a greater tendency towards perianal involvement*. It does not often occur before puberty, and a postmenopausal onset is rare’. There is no racial predilection”. A tropical environment favours the development of HS, but it has been seen in many parts of the world under varying climatic conditions’ Obesity and a genetic tendency to acne are apparent predisposing factor^".'^. Although HS is confined primarily to adults, a case has been described affecting the perineum of a 2-year-old child’’ and the disease has also been noted in a Shetland sheepdog bitchI4.
’.
Pathogenesis HS appears to be a poral occlusive disease with an added element of bacterial infection. The early sequential changes include first a keratinous plugging of the apocrine duct, then dilatation of the gland, €allowed by severe inflammation’ Bacteria, which gain access to the apocrine system via the follicle, are trapped beneath the keratinous plug and multiply
’.
ooO7-1323/92/090863-04
C
1992 Butterworth-Heinemann Ltd
rapidly in the nutrient milieu of the apocrine sweatI6. Gland rupture occurs, leading to extension of infection to adjacent apocrine glands’ ’. Superadded bacterial infection with streptococci and staphylococci may lead to further local extension, tissue destruction and skin damage’*. Fibrosis and sinus track formation result; these ultimately burn out, leaving damaged tissue that may subsequently flare up’’. Other apocrine areas, e.g. the opposite axilla and the anogenital and mammary areolar glands, may be involved either synchronously or later’’. Apocrine glands of the axillary skin” are confined mainly to the hair-bearing area and the surrounding 2 cm. The term ‘anal organ’ has been applied to the similarly concentrated mass of apocrine tissue situated in the circumanal region”. Adequate surgical excision of glands from these sites will be followed by a low recurrence rate. Apocrine glands are more diffuse in the groin, perineum and inframammary regions, making complete removal difficult; recurrence after surgery at these sites occurs more often. Recurrent disease may therefore reflect either too limited an excision or widespread, sometimes ‘ectopic’, gland distribution. Recurrence in the first instance usually requires further surgery to complete the excision; in the latter case it can be managed conservatively or by local removal of the recurrent lesion. This is discussed further under surgical treatment. The exact mechanisms predisposing to these events remain unclear but may involve one of several features. HS has been simulated by producing poral occlusion and added bacterial infection. However, it is unclear whether occlusion is primary or secondary”. HS is associated with various physiological hormonal events, e.g. onset of puberty and pregnancy, and is also commoner during the second half of the menstrual cycle, which implies that a high 0estrogen:androgen ratio may predisposez3. The disease is also seen in association with overt endocrine disorders such as diabetes, Cushing’s disease and a c r ~ m e g a l y However, ~ ~ ~ ~ ~ .a recent paper examining glucose tolerance, clinical features, microbiology, immunology and human leucocyte antigen frequencies failed to show any definite pattern in 27 patientsz6. It has also been suggested that the disease may be related to reduced cutaneous levels of the protective calcium-binding protein calprotectin, and also reduced zinc and ascorbate levels27.A high incidence of atopy, eczema and drug allergies has also been reported”. There is no significant difference in the size or density of the apocrine glands in patients with HS compared with controls, but the apocrine glands in those with axillary hyperhidrosis are significantly larger than in HS or controls”. HS is associated with other skin diseases that show poral occlusion, e.g. Fox-Fordyce diseasezg, pityriasis rubra
Hidradenitis suppurativa: A. K. Banerjee
2 per cent iodine-starch in alcohol ( 7 5 mg in 100 ml castor oil), blocking exocrine sweating with atropine (1.2 mg intravenously), and stimulating apocrine glands with oxytocin ( 2 units intravenously). The resulting gap in skin cover may be left to heal primarily by granulation". Silastic (Dow Corning, Reading, U K ) foam may be a useful adjunct to this process. Of 17 patients undergoing radical excision of HS whose wounds were allowed to heal by granulation, axillary excisions were performed in nine and perineal surgery in the remainder. Initially the cavity was packed with ribbon gauze soaked in a proflavine-liquid paraffin mixture. On the third day after operation Silastic foam dressing was applied. Axillary excisions required 50 ml and perineal excisions 100-200 ml Silastic mixture. Proponents of this technique claim that it permits adequate disease clearance and results in a cosmetically Complications acceptable scar, superior to that obtained by skin grafting and Folliculitis and other local or systemic infections resulting from with little limitation of movement. It also avoids the pain of the spread of bacteria, marked fibrosis of an infected area with conventional management of open granulating wounds by restricted mobility of the associated limb, anal and rectal gauze packing5'. fistulation in the perianal form of the disease and, rarely, Once the defect is bacteriologically clean, various methods squamous cell carcinoma of the skin may all occur34. Other may be used to facilitate healing (Table 1). Skin grafts have complications that have been reported include urethral, vesical been employed, the thigh being a common donor site. and rectal fistulae35, anaemia36, interstitial k e r a t i t i ~ ~ ~ , Rotational flaps and free flaps have also been used by plastic hypoproteinaemia, and amyloidosis, which may rarely progress However, the terms 'wide' and 'radical' excision to renal failure and death3'. are poorly defined in many papers, and so general comparison of the various methods is unsatisfactory. Emphasis is often Medical management placed on the technique used to cover an excision defect, rather than on the extent of excision or the success or failure of the Initial treatment comprises cleaning with ordinary soaps, treatment. Follow-up has been s h o d 3 or of unspecified careful drying, povidone-iodine (Betadine; Napp Laboratories, length64and often absent6s.66.Recurrence rates that are quoted Cambridge, U K ) and other antiseptics. Open, wet dressings without reference to the site at which surgery was performed and loose clothing are indicated39.Mild topical steroid creams may mislead63, and considering reoperation as a criterion of are of use, but are often more effective in combination with treatment failure will underestimate the number of patients with systemic antibiotics such as penicillin, erythromycin or a less than satisfactory outcome16. Table 1 gives a summary of t e t r a ~ y c l i n e ~This ~ ~ ~produces '. clearance of mild attacks in all series published in sufficient detail to allow meaningful about one-third of cases and can also stop initial disease comparison, although some data are either unavailable or progression. Topical antibiotics of the aminoglycoside group, unclear. Harrison et a1.62, in the most complete survey e.g. clindamycin, can also reduce cutaneous inflammation4'. available, reviewed 82 patients who had been treated by radical Intralesional triamcinolone may produce regression2. Control surgery (118 excisions) from 6 to 89 months after surgery. Local of HS has also been reported in mild to moderate cases in recurrence rates varied greatly with the disease site, being low women using combined antiandrogen (cyproterone acetate) after axillary ( 5 per cent) and perianal (nil) surgery and high and oestrogen therapy42. A separate double-blind controlled, after inguinoperineal ( 3 7 per cent ) and submammary cross-over trial comparing ethinyloestradiol 50 pg plus (50 per cent ) excision. Recurrence resulted mainly from cyproterone acetate 50 mg in a reverse sequential regimen with inadequate excision or an unusually wide distribution of ethinyloestradiol50 pg plus norgestrel500 pg showed that these apocrine glands, but physical factors such as obesity, local treatments were equally effective43, although only seven of 18 pressure and skin maceration also played a part in a few patients who completed the trial were free of disease at 18 patients. Recurrence resulting from inadequate surgery tended months' follow-up. Patients with established sepsis secondary to be the most troublesome and 25 per cent of patients to extensive hidradenitis responded less well, reinforcing the developed disease at a new anatomical site after operation. Two need for early diagnosis and treatment if conservative measures distinct patterns of recurrent disease were found. One was are to offer any chance of success. confined to the immediate vicinity of the scar, usually solitary Synthetic retinoids (acitretin) have also been successfully and typically occurring in the groin creases or the employed in clearing hidradenitis when applied topically, but posteroinferior margins after inguinoperineal surgery. The experience is limited44.4s and one trial failed to confirm any second, in which multiple lesions were present, occurred either advantage46. Combination hypothalamic-pituitary-ovarian adjacent to previous excision sites or a few centimetres away. axis and adrenal suppression has also been used with some The former, typified by deep sinuses and appreciable morbidity, benefit4'. Earlier studies failed to show any improvement from required further excision; the latter, characterized by stimulating the hypothalamoadrenal axis with polysaccharides widespread, often superficial lesions, was trivial and resulted in derived from bacteria, and even insulin and thyroxine little morbidity62.In this series, all complications were relatively administration have been tried unsuccessfully4'. minor. Failure of the skin graft, defined as
A. K. Banerjee Department of Surgery, Sandwell District General Hospital, Lyndon, West Bromwich, Birmingham B714HJ, UK Correspondence to: Mr A. K. Banerjee
Surgical treatment of hid radenitis su ppurat iva The management of hidradenitis suppurativa is frequently unsatisfactory. No method satisfies all requirements f o r the ideal treatment quick healing, no hospital admission, minimal patient inconvenience and low recurrence - but greater awareness of the strengths and weaknesses of existing methods should lead to improved management. Good reports of the relative cure rates of the diflerent surgical options are scarce and controlled trials non-existent, but existing evidence is reviewed critically and summarized. The operation of choice in most instances is wide local excision and healing .by secondary intention.
The ideal treatment of hidradenitis suppurativa ( H S ) should provide a high likelihood of cure with a low recurrence rate, should avoid hospital admission and general anaesthesia, and should involve minimal inconvenience and time off work for the patient. The disease shows a wide spectrum of severity; many cases remain mild and never progress beyond one site, and some undergo spontaneous remission. However, some untreated patients can be expected to progress relentlessly until total involvement of individual and additional apocrine areas is complete’. Properly diagnosed and adequately treated, early stages of the disease can often be controlled with medical measures. However, in established HS there is no evidence that treatment other than surgery has any effect on the natural history of the condition. As the disease becomes more chronic, obtaining complete resolution is increasingly difficult and in advanced cases may be impossible’. HS is a chronic, suppurative and cicatricial disease of apocrine gland-bearing skin areas, principally the axillae, anogenital skin and, rarely, the breasts. The disease was first documented in 1839 by Velpeau3, who reported a peculiar localization of cutaneous abscesses to the axillary, mammary and perianal skin; both Lane4 in 1933 and Brunsting’ in 1939 described the clinical features of the disease more fully and its frequent association with acne. Shelley and Cahn demonstrated an experimental model of the disease6.
Incidence HS occurs in both sexes7, although women develop the axillary form more frequently, while men show a greater tendency towards perianal involvement*. It does not often occur before puberty, and a postmenopausal onset is rare’. There is no racial predilection”. A tropical environment favours the development of HS, but it has been seen in many parts of the world under varying climatic conditions’ Obesity and a genetic tendency to acne are apparent predisposing factor^".'^. Although HS is confined primarily to adults, a case has been described affecting the perineum of a 2-year-old child’’ and the disease has also been noted in a Shetland sheepdog bitchI4.
’.
Pathogenesis HS appears to be a poral occlusive disease with an added element of bacterial infection. The early sequential changes include first a keratinous plugging of the apocrine duct, then dilatation of the gland, €allowed by severe inflammation’ Bacteria, which gain access to the apocrine system via the follicle, are trapped beneath the keratinous plug and multiply
’.
ooO7-1323/92/090863-04
C
1992 Butterworth-Heinemann Ltd
rapidly in the nutrient milieu of the apocrine sweatI6. Gland rupture occurs, leading to extension of infection to adjacent apocrine glands’ ’. Superadded bacterial infection with streptococci and staphylococci may lead to further local extension, tissue destruction and skin damage’*. Fibrosis and sinus track formation result; these ultimately burn out, leaving damaged tissue that may subsequently flare up’’. Other apocrine areas, e.g. the opposite axilla and the anogenital and mammary areolar glands, may be involved either synchronously or later’’. Apocrine glands of the axillary skin” are confined mainly to the hair-bearing area and the surrounding 2 cm. The term ‘anal organ’ has been applied to the similarly concentrated mass of apocrine tissue situated in the circumanal region”. Adequate surgical excision of glands from these sites will be followed by a low recurrence rate. Apocrine glands are more diffuse in the groin, perineum and inframammary regions, making complete removal difficult; recurrence after surgery at these sites occurs more often. Recurrent disease may therefore reflect either too limited an excision or widespread, sometimes ‘ectopic’, gland distribution. Recurrence in the first instance usually requires further surgery to complete the excision; in the latter case it can be managed conservatively or by local removal of the recurrent lesion. This is discussed further under surgical treatment. The exact mechanisms predisposing to these events remain unclear but may involve one of several features. HS has been simulated by producing poral occlusion and added bacterial infection. However, it is unclear whether occlusion is primary or secondary”. HS is associated with various physiological hormonal events, e.g. onset of puberty and pregnancy, and is also commoner during the second half of the menstrual cycle, which implies that a high 0estrogen:androgen ratio may predisposez3. The disease is also seen in association with overt endocrine disorders such as diabetes, Cushing’s disease and a c r ~ m e g a l y However, ~ ~ ~ ~ ~ .a recent paper examining glucose tolerance, clinical features, microbiology, immunology and human leucocyte antigen frequencies failed to show any definite pattern in 27 patientsz6. It has also been suggested that the disease may be related to reduced cutaneous levels of the protective calcium-binding protein calprotectin, and also reduced zinc and ascorbate levels27.A high incidence of atopy, eczema and drug allergies has also been reported”. There is no significant difference in the size or density of the apocrine glands in patients with HS compared with controls, but the apocrine glands in those with axillary hyperhidrosis are significantly larger than in HS or controls”. HS is associated with other skin diseases that show poral occlusion, e.g. Fox-Fordyce diseasezg, pityriasis rubra
Hidradenitis suppurativa: A. K. Banerjee
2 per cent iodine-starch in alcohol ( 7 5 mg in 100 ml castor oil), blocking exocrine sweating with atropine (1.2 mg intravenously), and stimulating apocrine glands with oxytocin ( 2 units intravenously). The resulting gap in skin cover may be left to heal primarily by granulation". Silastic (Dow Corning, Reading, U K ) foam may be a useful adjunct to this process. Of 17 patients undergoing radical excision of HS whose wounds were allowed to heal by granulation, axillary excisions were performed in nine and perineal surgery in the remainder. Initially the cavity was packed with ribbon gauze soaked in a proflavine-liquid paraffin mixture. On the third day after operation Silastic foam dressing was applied. Axillary excisions required 50 ml and perineal excisions 100-200 ml Silastic mixture. Proponents of this technique claim that it permits adequate disease clearance and results in a cosmetically Complications acceptable scar, superior to that obtained by skin grafting and Folliculitis and other local or systemic infections resulting from with little limitation of movement. It also avoids the pain of the spread of bacteria, marked fibrosis of an infected area with conventional management of open granulating wounds by restricted mobility of the associated limb, anal and rectal gauze packing5'. fistulation in the perianal form of the disease and, rarely, Once the defect is bacteriologically clean, various methods squamous cell carcinoma of the skin may all occur34. Other may be used to facilitate healing (Table 1). Skin grafts have complications that have been reported include urethral, vesical been employed, the thigh being a common donor site. and rectal fistulae35, anaemia36, interstitial k e r a t i t i ~ ~ ~ , Rotational flaps and free flaps have also been used by plastic hypoproteinaemia, and amyloidosis, which may rarely progress However, the terms 'wide' and 'radical' excision to renal failure and death3'. are poorly defined in many papers, and so general comparison of the various methods is unsatisfactory. Emphasis is often Medical management placed on the technique used to cover an excision defect, rather than on the extent of excision or the success or failure of the Initial treatment comprises cleaning with ordinary soaps, treatment. Follow-up has been s h o d 3 or of unspecified careful drying, povidone-iodine (Betadine; Napp Laboratories, length64and often absent6s.66.Recurrence rates that are quoted Cambridge, U K ) and other antiseptics. Open, wet dressings without reference to the site at which surgery was performed and loose clothing are indicated39.Mild topical steroid creams may mislead63, and considering reoperation as a criterion of are of use, but are often more effective in combination with treatment failure will underestimate the number of patients with systemic antibiotics such as penicillin, erythromycin or a less than satisfactory outcome16. Table 1 gives a summary of t e t r a ~ y c l i n e ~This ~ ~ ~produces '. clearance of mild attacks in all series published in sufficient detail to allow meaningful about one-third of cases and can also stop initial disease comparison, although some data are either unavailable or progression. Topical antibiotics of the aminoglycoside group, unclear. Harrison et a1.62, in the most complete survey e.g. clindamycin, can also reduce cutaneous inflammation4'. available, reviewed 82 patients who had been treated by radical Intralesional triamcinolone may produce regression2. Control surgery (118 excisions) from 6 to 89 months after surgery. Local of HS has also been reported in mild to moderate cases in recurrence rates varied greatly with the disease site, being low women using combined antiandrogen (cyproterone acetate) after axillary ( 5 per cent) and perianal (nil) surgery and high and oestrogen therapy42. A separate double-blind controlled, after inguinoperineal ( 3 7 per cent ) and submammary cross-over trial comparing ethinyloestradiol 50 pg plus (50 per cent ) excision. Recurrence resulted mainly from cyproterone acetate 50 mg in a reverse sequential regimen with inadequate excision or an unusually wide distribution of ethinyloestradiol50 pg plus norgestrel500 pg showed that these apocrine glands, but physical factors such as obesity, local treatments were equally effective43, although only seven of 18 pressure and skin maceration also played a part in a few patients who completed the trial were free of disease at 18 patients. Recurrence resulting from inadequate surgery tended months' follow-up. Patients with established sepsis secondary to be the most troublesome and 25 per cent of patients to extensive hidradenitis responded less well, reinforcing the developed disease at a new anatomical site after operation. Two need for early diagnosis and treatment if conservative measures distinct patterns of recurrent disease were found. One was are to offer any chance of success. confined to the immediate vicinity of the scar, usually solitary Synthetic retinoids (acitretin) have also been successfully and typically occurring in the groin creases or the employed in clearing hidradenitis when applied topically, but posteroinferior margins after inguinoperineal surgery. The experience is limited44.4s and one trial failed to confirm any second, in which multiple lesions were present, occurred either advantage46. Combination hypothalamic-pituitary-ovarian adjacent to previous excision sites or a few centimetres away. axis and adrenal suppression has also been used with some The former, typified by deep sinuses and appreciable morbidity, benefit4'. Earlier studies failed to show any improvement from required further excision; the latter, characterized by stimulating the hypothalamoadrenal axis with polysaccharides widespread, often superficial lesions, was trivial and resulted in derived from bacteria, and even insulin and thyroxine little morbidity62.In this series, all complications were relatively administration have been tried unsuccessfully4'. minor. Failure of the skin graft, defined as
