Cerebral vasculitis in a case of henoch–Schönlein purpura: An unusual presentation

Abstract

Background: Henoch–Schonlein purpura (HSP) is the most common vasculitis seen in children that rarely involve central nervous system (CNS) (0.65%–8%). Patients with CNS vasculitis may present with symptoms such as headache, seizures, focal neurological deficit, or behavior abnormalities. We describe a case of HSP where the patient presented with developmental delay along with skin rash and renal involvement. Clinical Description: A 15-month-old boy presented with a history of developmental delay and skin rashes for the past 2 months. There was no significant antenatal or birth history. At the age of 15 months, the child could not sit without support, could not transfer objects from one hand to other, could only speak bisyllables, and could not wave “bye-bye.” On examination, the patient had normal vitals with equal volume peripheral pulses and normal blood pressure in all four limbs. Urine examination showed microscopic hematuria, and kidney functions were mildly deranged. Skin biopsy showed immunoglobulin A vasculitis. Magnetic resonance imaging brain was done which revealed multifocal areas of altered signal intensity in periventricular deep white matter in bilateral frontal and parieto-occipital lobes, appearing hyperintense on T2-weighted and fluid-attenuated inversion recovery sequences, showing evidence of diffusion restriction, suggestive of areas of acute ischemia. The findings were consistent with manifestations of CNS vasculitis. Management: The patient was managed conservatively and advised physiotherapy and follow-up for developmental delay. Conclusion: HSP can rarely involve CNS, however, developmental delay in HSP has never been reported before.