Pathophysiology
No studies investigating the size and secretion rate of the apocrine glands in health and disease are known to this author. The existence of plugged follicles in hidradenitis suppurativa has been accepted histologically since Shelley and Cahn's original work on the pathogenesis of the condition. More recently Highet et al and Mortimer have stressed the common occurrence of comedones in hidradenitis suppurativa but their production and relationship to inflamed lesions is not known. Microbiological analysis of hidradenitis suppurativa gives no consistent results. In early disease no pathogens are identified but, given the appropriate environment, the normal skin flora could, as in acne, mediate inflammation. There is clearly scope for much research.
The most recently investigated aspect of hidradenitis suppurativa is systemic hormone levels: plasma testosterone (as measured by the free androgen index, for example) is significantly higher in patients compared to controls though there was considerable overlap between some patients and controls. These data suggest that plasma androgens are permissive and that there may be end-organ hyperresponsiveness which explains the disease and its severity.
Dvorak et al found that in hidradenitis suppurativa no abnormality was demonstrated in any granulocyte or cell-mediated immune function tests. All patients had normal immunoglobulin levels and elevated total haemolytic complement. It was concluded that hidradenitis suppurativa is a localized chronic inflammation of apocrine glands without a generalized defect in host defence.
Mortimer concluded that patients with hidradenitis suppurativa appear to have endocrine abnormalities sufficient to suggest an androgenic basis for this disease.31 In this way hidradenitis suppurativa falls into the same category as acne vulgaris and idiopathic hirsutism: endocrinologically representing a heterogeneous group, with no single endocrinological abnormality determinable in every patient. Treatment Diagnosis and treatment before undermining sinus tracts and burrowing abscesses have developed is crucial for satisfactory control of the disease. Identification of comedones in affected and nonaffected apocrine sites, as well as retro-auricular areas, is important for mni early recognition. The response to treatment in the early or intermediate stages with longterm antibiotics (six months or more) is variable and at times frankly disappointing. Nevertheless, they should be tried. Tetracycline (1 g/day), minocycline (100 mg/day) or clindamycin (600 mg/day) being the drugs of choice.
An exciting new development for females with hidradenitis suppurativa is the use of anti-androgens .39'40 In order to examine whether antiandrogen therapy was effective in hidradenitis suppurativa, ethinyloestradiol 50 i^g/cyproterone acetate 50 mg in a reverse sequential regimen was compared with ethinyloestradiol 50 (j,g/levonorgestrel 250 |xg (Eugynon 50) in twenty-four female patients. Both treatments produced substantial improvement in disease activity: overall, 38 per cent of patients were cleared and have remained free of disease for eighteen months; 29 per cent of patients improved; 20 per cent remained unchanged; while 12 per cent deteriorated. Clinically, cyproterone acetate was not significantly more effective than Eugynon 50. This author's department has also used, in an uncontrolled study, spironolactone - up to 200 mg/day - and obtained some clinical benefit in about 50 per cent of patients. Unfortunately, these hormonal regimens have too many anti-androgenic side-effects to be given to males.
The role of isotretinoin in hidradenitis suppurativa is controversial but most centres consider it of little or no benefit except possibly in doses of 2 mg/kg/day for even up to two years. However, Boer concluded that the use of retinoids should be considered even though the response rate is only 30-50 per cent.41 He suggested that another approach might be to combine isotretinoin therapy with other forms of treatment such as oral antibiotics, oral corticosteroids or intralesional corticosteroid injections.
Data presented by Hughes (personal communication 1988) based on a survey of 150 patients with hidradenitis suppurativa indicate that surgery is unquestionably of value in patients with moderate to severe disease. Excision of the area affected (plus a 2 cm rim of normal tissue) in the axilla and perineum is of considerable benefit. In the perimammary area wide excision of the individual lesions is preferable. Healing by granulation tissue is preferred to grafting but occasionally mesh grafts are indicated.
Thus, hidradenitis suppurativa often benefits from early diagnosis and good collaboration between surgeons and dermatologists in an attempt to control what can be, at times, a physically and psychologically devastating disease.