A 16-year-old boy presented with intermittent scalp crusting and alopecia. Six months previously, he had presented with abdominal pain, diarrhoea and anaemia, and at that time, had also been found… Click to show full abstract
A 16-year-old boy presented with intermittent scalp crusting and alopecia. Six months previously, he had presented with abdominal pain, diarrhoea and anaemia, and at that time, had also been found to have aortic regurgitation. Extensive gastrointestinal investigations at that time were negative. On physical examination, the patient was found to have unilateral, heavily impetiginized scalp crusting (Fig. 1a) and secondary scarring alopecia. The patient presented again 9 months later with bilateral scalp crusting and associated spiking fevers, lethargy and weight loss. Repeat blood investigations showed a drop in haemoglobin from 120 g/L to 85 g/L (normal range) along with raised C-reactive protein (140 mg/L; normal <10 mg/L) and erythrocyte sedimentation rate (125 mm/h; normal range < 0–15 mm/h, respectively). Blood pressure was unrecordable, and there was absence of the left radial pulse. On further examination, a collapsing pulse, harsh diastolic murmur, left carotid bruit and a ‘pistol shot’ femoral bruit were found. Echocardiography and cardiac magnetic resonance imaging demonstrated dilation of the ascending aorta and severe aortic regurgitation. Fluorodeoxyglucose– positron emission tomography scan (FDG-PET) revealed appearances consistent with aortitis and large-vessel vasculitis (Fig. 2). Histopathological findings
               
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