Q J Med 2002; 95: 636-637
© 2002 Association of Physicians
Correspondence |
A recurrent rash treated by oophorectomy
1 Department of Dermatology 2 Department of Endocrinology, University Hospitals Birmingham NHS Trust
Sir,
A 45-year-old woman first presented in 1976 with a 5-year history of a pruritic rash on the elbows and dorsae and palms of both hands since menarche. This was accompanied by oral and sometimes genital ulceration, which required treatment with prednisolone 30 mg/day when severe. The oral ulceration was often of sufficient severity to prevent her from eating, and at age 25, she was investigated for weight loss; these investigations were normal. At age 20, she had started the combined oral contraceptive pill and remained on this for 10 years, but continued to require short courses of steroids every month.
The rash comprised erythematous papules and target-like lesions, distributed symmetrically on the hands and elbows, with haemorrhagic crusting and fissuring of her lip following blistering. Each episode began one week before menstruation and remitted afterwards.
Histology of a typical lesion showed a dermal inflammatory infiltrate with necrosis of keratinocytes in the epidermis, consistent with erythema multiforme (EM).
Many cases of EM, including cyclical EM, are secondary to Herpes simplex infection,1 which may be subclinical. There was no history or clinical evidence of H. simplex infection in our patient and long-term acyclovir as empirical treatment reduced her prednisolone requirement slightly but without significant symptomatic benefit. Azathioprine, dapsone and thalidomide were all tried in succession with little or no benefit.
Due to the cyclical nature of her EM, the disability caused by her mouth ulcers and the failure of standard treatments, medical ‘oophorectomy’ was induced with the gonadotrophin-releasing hormone agonist, Buserelin, via nasal spray, 300 mcg bd. This resulted in complete remission and obviated her requirement for steroids. After 6 months of treatment and extensive counselling, she underwent oophorectomy and remained symptom-free. However, with the development of menopausal symptoms the patient commenced Saw Palmetto, an over-the-counter herbal anti-androgen preparation. Both this and subsequent treatment with Prempak C, a combined hormone replacement therapy, provoked recurrence of oral and genital ulceration.
Skin challenge tests with a 25 mcg oestrogen patch and an intradermal progestogen prick test (medroxyprogesterone acetate) showed no immediate reaction, but erythema and swelling was seen at the progestogen site 24 h later.
Because of menopausal symptoms and risk of osteoporosis, exacerbated by recurrent prednisolone treatment, she is now taking unopposed oestrogen HRT with transvaginal ultrasound monitoring and biopsy of her endometrium.
The spectrum of EM ranges from mild rash to life-threatening blistering and ulceration (Stevens-Johnson syndrome). The rash typically develops over a few days and may or may not show target features of different zones of erythema. The diagnosis is made clinically and by characteristic histology. EM generally resolves within a few days, but recurrent attacks are well recognized. Some 70% of cases are associated with H. simplex, which may be facial, genital or sub-clinical; viral antigens have been demonstrated in skin biopsies of some patients.2
CD4+ and CD8+ lymphocytes can also be seen on histology and the condition is thought to be immune mediated, probably a delayed-type hypersensitivity, though immune complexes have been shown in some patients. There are many other rare causes of EM (Table 1
), implying a range of triggering antigens.
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Auto-immune progesterone dermatitis (AIPD) is a group of disorders first described in 1964.3 It is defined as a skin condition regularly appearing pre-menstrually and settling with the onset of menses, in other words associated with the post-ovulatory rise in serum progesterone. Recognized dermatoses which can behave in this way include eczema, pompholyx, urticaria and EM. Hypersensitivity to progesterone can be shown by the presence of auto-antibodies4 or, as demonstrated in this case, through a challenge test. The timing of onset of the rash in this case, together with its exacerbation following pharmacological progestins and suppression following ablation of ovulation and subsequent oophorectomy strongly suggests an aetiological role for progesterone.
References
1. Leigh IM, Mowbray JF, Levene GM, et al. Recurrent and continuous erythema multiforme: a clinical and immunological study. Clin Exp Dermatol1985; 10:58–67.[Web of Science][Medline]
2. McDonald A, Feiwel M. Isolation of herpes simplex virus from erythema multiforme. Br Med J1972; 2:570.
3. Shelley WB, Preucel RW, Spoont SS. Autoimmune progesterone dermatitis. JAMA1964; 190:147–50.
4. Pinto JS, Sobrinho L, da Silva MB, et al. Erythema multiforme associated with autoreactivity to 17 alpha-hydroxyprogesterone. Dermatologica1990; 180:146–50.[Medline]
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