Posterior reversible encephalopathy syndrome in the setting of IgA vasculitis

Noah Fanous; Emily Clarke; Moise L Levy

doi:10.1111/pde.15569

Posterior reversible encephalopathy syndrome in the setting of IgA vasculitis

Pediatr Dermatol. 2024 Feb 18. doi: 10.1111/pde.15569. Online ahead of print.

Authors

Noah Fanous¹, Emily Clarke², Moise L Levy^{2

3}

Affiliations

¹ The University of Texas Health Sciences Center at San Antonio, San Antonio, Texas, USA.
² Division of Dermatology, Dell Medical School at the University of Texas at Austin, Austin, Texas, USA.
³ Department of Pediatrics, Dell Medical School at the University of Texas at Austin, Austin, Texas, USA.

PMID: 38368940
DOI: 10.1111/pde.15569

Abstract

IgA vasculitis (IgAV), formerly known as Henoch-Scholein purpura, is a small vessel vasculitis, most commonly seen in pediatric patients, that can affect numerous internal organs including the kidneys, lungs, gastrointestinal tract, and the central nervous system (CNS). CNS manifestations of this condition include hypertensive encephalopathy, thrombosis, optic neuropathy, seizures, CNS vasculitis, and a more recently described phenomenon known as posterior reversible encephalopathy syndrome (PRES). Symptoms of PRES include hypertension, altered mental status, and seizures caused by vasogenic disruption of the blood-brain barrier, and the condition is diagnosed by characteristic edema-related gray-white matter changes in the parieto-occipital lobes on magnetic resonance imaging. Herein, we present a rare case of PRES as a presenting sign of IgAV to increase awareness about this unusual association.

Keywords: HSP; Henoch-Schonlein purpura; IgA vasculitis; IgAV; PRES; posterior reversible encephalopathy syndrome; vasculitis.

Publication types

Letter