Case report

A 25 year old woman presented to her general practitioner with an urticarial rash on her chest. Three weeks later it had spread to her cheeks and forehead, but sparing the eyelids, to the shoulders and dorsum of the hands. This was associated with increasing fatigue, mouth ulceration, peri-ungual erythema, and arthralgia of the wrists and ankles. She had been previously healthy, was taking no medication and the family history was unremarkable. A diagnosis of systemic lupus erythematosus (SLE) was made by a dermatologist and oral prednisolone was started at 40 mg/day.

Investigations were as follows: haemoglobin 14.2 g/dl, leucocytes 6.4 × 10⁹/l, platelets 100 × 10⁹/l, erythrocyte sedimentation rate (ESR) 7 mm 1st h, antinuclear antibody (ANA), deoxyribonucleic acid (DNA), anti-cardiolipin antibodies, rheumatoid factor (RF), and extractable nuclear antigens (ENA), including Jo-1, were all negative. Immunoglobulin concentrations were normal. One week later, because of worsening myalgia, the creatine kinase (CK) was measured and found to be increased at 2999 iu/l (normal < 160 iu/l). The revised diagnosis was dermatomyositis.

The patient was admitted to hospital for observation and the dose of oral prednisolone increased to 60 mg/day. Azathioprine was started at 50 mg/day. On the second day of admission, four weeks after the onset of her systemic symptoms, she complained of visual blurring. The Snellen visual acuity was 6/24 in both eyes. Scattered posterior pole cotton wool spots and haemorrhages with macular oedema were reported by an ophthalmologist. The CK had risen to 6650 iu/l. Chest radiograph, electrocardiogram, and renal function were normal. Pulsed intravenous methylprednisolone at a dose of 500 mg for three days was given at this stage, in view of the acute general deterioration. Within a week of admission, she was transferred to Leeds …