Cutaneous vasculitis and collapse.

Accepted 23 April 1998 A 20-year-old man was admitted with a 1-week history of a vasculitic rash over both legs and aching in both calves. He felt otherwise well although he had suffered an upper respiratory tract infection 2 weeks previously, manifesting as a mild sore throat for 1 day. Apart from a small ventriculo-septal defect (VSD) reviewed annually, he had no significant medical or dental history and took no medications. He gave no history of intravenous drug abuse. General physical examination was unremarkable. In particular, there was no lymphadenopathy or splenomegaly and he was consistently apyrexial. A long-standing pansystolic murmur, grade 3/6, was confirmed at the cardiac apex, heart rate was 70 in sinus rhythm and blood pressure was 120/70 mmHg. Admission investigations including full blood count, antinuclear antibodies, complement, renal and liver function tests, were normal. A throat swab was negative for bacterial growth and an anti-streptolysin-O (ASO) titre was within normal limits. The erythrocyte sedimentation rate (ESR) was elevated at 45 mm/h and immune complexes were detectable in serum. Antineutrophil cytoplasmic antibodies, cytoplasmic pattern (cANCA) were detected by both indirect immunofluorescence on whole neutrophils and using a proteinase 3 ELISA. There was blood and protein detectable in the urine although it was negative for casts and bacterial growth. These findings were consistent with a diagnosis of small vessel vasculitis and he was commenced on oral prednisolone 35 mg daily and topical betamethasone cream 0.1%. Skin biopsy subsequently showed only non-specific mild inflammation and direct immunofluorescence examination of a skin specimen was normal. The rash settled rapidly allowing cessation of his treatment, but he was admitted acutely, 5 weeks after the onset of symptoms, with collapse secondary to a sustained tachyarrhythmia and with a florid recrudescence of his rash (figure 1). On admission, his heart rate and blood pressure were normal and auscultation confirmed the pansystolic murmur as before. He was now pyrexial with a temperature of 38°C. A transthoracic echocardiogram was performed (figure 2).