Clinical dermatology • Concise report Paper No.: 451

Severe vulval apocrine acne successfully treated with prednisolone and isotretinoin

L. A. Fearfield and R. C. D. Staughton Department of Dermatology, Chelsea and Westminster Hospital, Imperial College School of Medicine, London, UK

Summary Apocrine acne, otherwise known as hidradenitis suppurativa, is a chronic inflammatory scarring disease affecting the apocrine gland-bearing skin. We present a case of a 34- year-old woman with severe vulval apocrine acne who was successfully treated initially with prednisolone and then maintained on long-term isotretinoin. This case indicates that long-term treatment with isotretinoin may be more successful than the usual 4–6 months treatment regime.

A 34-year-old woman presented 8 weeks post partum labia majora showed two densely inflamed hair follicles with such severe and painful vulval inflammation that bridged by aggregates of neutrophils forming micro- she was unable to walk or sleep. Vulval ‘boils’ had begun abscesses. Immunofluorescence was negative, as were 2 years previously during her second pregnancy. Despite tissue bacterial and fungal cultures. numerous courses of antibiotics and topical treatments Our current regime for the management of violently including potassium permanganate soaks, metronidazole purulent cystic acne (acne fulminans and pyoderma 0.75% gel (metrogel), mupirocin 2% ointment (bactro- faciale) comprises pretreatment with oral steroids and ban) and various antiseptics, the problem had persisted. anti-inflammatory antibiotics, followed by the gradual There was a past history of scarring acne, quiescent for introduction of isotretinoin and later the cautious with- the last 6 years. drawal of steroids. We embarked on this same course Examination revealed multiple pustular and cystic with our patient and Fig. 2 illustrates diagramatically lesions located on swollen red labia majora (Fig. 1). A the gratifying response. Prednisolone 20 mg per day and few cystic lesions were noted in both axillae and behind erythromycin 500 mg four times per day were com- the ears. A diagnosis of apocrine acne (hidradenitis menced. After four weeks the prednisolone was gradually suppurativa) was made. In view of the intense discomfort reduced; 10 mg/day for 7 weeks, 5 mg/day for 12 weeks and difficulty in walking the patient was admitted to and 2.5 mg/day for 4 weeks. The erythromycin was also hospital. tailed off after 4 weeks to 500 mg/day for a further Full blood count and glucose were normal. The 8 weeks. Isotretinoin 20 mg/day (0.33 mg/kg/day) was erythrocyte sedimentation rate (20 mm/h) and C-reactive introduced after 8 weeks of prednisolone and erythro- protein (18 mg/L) were raised. Swabs from the vulval mycin. After 4 weeks this was increased to 40 mg/day lesions grew a mixture of diphtheroids, Proteus, lactose (0.66 mg/kg/day) and further increased to 60 mg/day fermenting coliform, Streptococcus faecalis and coagulase- (0.98 mg/kg/day). This was continued for 12 months negative Staphylococcus.NoStreptococcus milleri were and by the end of this period the vulva was clear of found.1 A biopsy taken from a purulent lesion on the inflammation. However, 1 month after discontinuation of the isotretinoin one or two cystic lesions erupted on the labia majora and therefore isotretinoin 60 mg/day

Correspondence: L. Fearfield, Dept Of Dermatology, Chelsea and was restarted and continued for a further 3 months. No Westminster Hospital, 369 Fulham Rd, London SW10 9NH, UK. significant relapse of the vulval apocrine acne has Tel.: þ44 181 7468167. Fax: þ44 181 7468578. occurred during the last 10 months after finally discon- Accepted for publication 12 November 1998 tinuing the isotretinoin.

᭧ 1999 Blackwell Science Ltd • Clinical and Experimental Dermatology, 24, 189–192 189 Successful treatment of vulval apocrine acne • L. A. Fearfield and R. C. D. Staughton

Hidradenitis suppurativa is a chronic, indolent con- dition characterized by purulent inflammation in apocrine glands2 and is thus better called apocrine acne. Lesions form in the apocrine gland-bearing skin of the axillae, the anogenital area, the areolae and behind the ears. The disease rarely begins before puberty when apocrine gland activity is minimal.3 Bridged comedones are the hall- mark, and multiple abscesses may develop frequently progressing to form sinus tracts. As the disease becomes chronic, extensive scarring and fibrosis occurs. With the resultant pain, limitation of movement and offensive chronic discharge, the patients’ quality of life can be severely compromised. The sexes are equally affected, although axillary disease is more common in women and anogenital disease in men.3 There is often a past history of severe acne and many patients, especially women, are obese.4 The most serious but rare long-term complica- tion is of squamous cell carcinoma, mostly occurring in men in the anogenital area. The time interval between diagnosis of apocrine acne and appearance of squamous cell carcinoma is, on average 19 years.5,6 The pathogenesis of apocrine acne is still debated. The anatomical distribution suggests that the disease is a primary disorder of apocrine glands and it has been suggested that follicular occlusion is the primary event. This causes obstruction to apocrine secretion as the ducts of apocrine glands drain into the main follicle. Histo- logical examination confirms ductal hypercornification Figure 1 Swollen labia majora with multiple cystic and pustular and distension of apocrine glands with surrounding lesions. purulent inflammation. The observed association with other conditions in which follicular occlusion is a pro- minent feature (acne vulgaris, acne conglobata, dissect- ing cellulitis of the scalp and pilonidal sinus) supports this view.2,7,8

Figure 2 Illustration of the good clinical response mapped against length of treatment.

190 ᭧ 1999 Blackwell Science Ltd • Clinical and Experimental Dermatology, 24, 189–192 Successful treatment of vulval apocrine acne • L. A. Fearfield and R. C. D. Staughton

There is evidence that androgens may play a permissive References role in the aetiology of apocrine acne.9 Apocrine acne is 1 Highet AS, Warren RE, Staughton RCD, Roberts SOB. associated with physiological endocrine events such as Streptococcus milleri causing treatable infection in perineal the onset of puberty, pregnancy and pre-menstrual hidradenitis suppurativa. Br J Dermatol 1980; 103: exacerbations. It has been reported as a presenting feature 375–82. 10 of premature adrenarche in a 7.8-year-old girl. Anti- 2 Attanoos R, Appleton M, Douglas-Jones A. The patho- androgen treatment has been used with modest success; genesis of hidradenitis suppurativa: a closer look at however, a recent study by Barth et al.11 found no apocrine and apoeccrine glands. Br J Dermatol 1995; supporting evidence of biochemical hyperandrogenism 133: 254–8. in women with apocrine acne. 3 Radcliffe K. Hidradenitis suppurativa. Genitourin Med Surgical removal of areas of burrowing inflammation 1991; 67: 58. offers a prompt solution to limited disease; however, 4 Banerjee A. Surgical treatment of hidradenitis suppura- tiva. Br J Surg 1992; 79: 863–6. more radical surgery may be needed for extensive dis- 5 Williams S, Busby R, DeMuth R, Nelson H. Perineal ease. Indeed it has been suggested previously that only 4,12,13 hidradenitis suppurativa: presentation of two unusual surgery offered a hope of a ‘cure’. Topical treat- complications and a review. Ann Plast Surg 1991; 26: ments including antiseptics, antibiotics and steroids are 456–62. usually only of limited benefit. Apocrine acne shows a 6 Mendonca H, Rebelo C, Fernandes A et al. Squamous cell much less favourable response to systemic antibiotics carcinoma arising in hidradenitis suppurativa. J Dermatol than acne vulgaris. Anti-androgen treatment with Surg Oncol 1991; 17: 830–2. cyproterone acetate in combination with ethinyloestra- 7 Ostlere L, Langtry J, Mortimer P, Staughton RCD. Hidra- diol in women has been used with some success.14,15 denitis suppurativa in Crohn’s disease. Br J Dermatol Systemic steroids quell purulent inflammation and are of 1991; 125: 384–6. particular use in the acute stages. 8 Yu C-W, Cook M. Hidradenitis suppurativa: a disease of follicular epithelium, rather than apocrine glands. Br J The retinoid drugs which are so dramatically success- Dermatol 1990; 122: 763–9. ful for sebaceous acne are less beneficial in this condition. 9 Ebling F. Hidradenitis suppurativa: an androgen-depen- This is probably because they are abandoned too soon; dent disorder. Br J Dermatol 1986; 115: 259–62. the inflammation is deeper and so more prolonged treat- 10 Lewis F, Messenger A, Wales J. Hidradenitis suppurativa ment is necessary. If successful, further treatment appears as a presenting feature of premature adrenarche. Br J 16–19 to be unecessary. Chow et al. have reported that Dermatol 1993; 129: 447–8. etretinate and acitretin are more effective than isotre- 11 Barth JH, Layton AM, Cunliffe WJ. Endocrine factors in tinoin in the treatment of apocrine acne.20 There have pre- and postmenopausal women with hidradenitis sup- been nine reported cases of successful treatment with purativa. Br J Dermatol 1996; 134: 1057–9. acitretin and etretinate, five of which had been previously 12 Bell B. Hidradenitis suppurativa. J Roy Soc Med 1978; 71: unresponsive to isotretinoin.20,21 The largest study by 511–5. 13 Palettta C, Jurkiewicz M. Hidradenitis suppurativa. Clin Stewart et al. involved six patients with apocrine acne Plast Surg 1987; 14: 383–90. who were treated with etretinate 0.35–1.1 mg/kg/day 14 Mortimer P, Dawber R, Gales M et al. A double-blind for up to 39 months. All patients demonstrated an controlled crossover trial of cyproterone acetate in females excellent response after 12 months; however, relapse with hidradenitis suppurativa. Br J Dermatol 1986; 115: occurred 4 months after stopping the treatment. It was 259–62. commented that no improvement was to be expected 15 Sawers R, Randall V, Ebling F. Control of hidradenitis until at least 6 months after starting treatment.17 suppurativa in women using combined antiandrogen The mechanisms of action of the retinoids in the (cyproterone acetate) and oestrogen therapy. Br J Der- treatment of apocrine acne may be multiple. Apocrine matol 1986; 115: 269–74. glands shrink with consequent reduction of secretions. 16 Jones D, Cunliffe W, King K. Hidradenitis suppurativa- Ductal hypercornification is reduced because of the influ- lack of success with 13-cis-retinoic acid. Br J Dermatol 1982; 107: 252. ence on keratinization. An antineutrophil effect also 17 Norris J, Cunliffe W. Failure of treatment of widespread occurs.17,22 The fact that some patients fail to respond hidradenitis suppurativa with isotretinoin. Clin Exp Der- to isotretinoin yet do so to etretinate and acitretin suggests matol 1986; 11: 579–83. that suppression of hyperkeratinization is more important 18 Dicken C, Powell S, Spear K. Evaluation of isotretinoin than glandular shrinkage. However, in parous women a treatment of hidradenitis suppurativa. J Am Acad Dermatol prolonged course of isotretinoin is probably a safer initial 1984; 11: 500–2. choice. 19 Brown C, Gallup D, Brown V. Hidradenitis suppurativa of

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the anogenital region: response to isotretinoin. Am J 21 Hogan D, Light M. Successful treatment of hidradenitis Obstet Gynaecol 1988; 158:12–5. suppurativa with acitretin. J Am Acad Dermatol 1988; 19: 20 Chow E, Mortimer P. Succesful treatment of hidradenitis 355–6. suppurativa and retroauricular acne with etretinate. Br J 22 Norris J, Cunliffe W. The effect of isotretinoin on apocrine Dermatol 1992; 126: 415–9. glands. Br J Dermatol 1988; 118: 295.

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