The University of Texas Health Sciences Center at San Antonio, San Antonio, Texas, USA.
Division of Dermatology, Dell Medical School at the University of Texas at Austin, Austin, Texas, USA.
Pediatr Dermatol. 2024 Jul-Aug;41(4):728-730. doi: 10.1111/pde.15569. Epub 2024 Feb 18.
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.
IgA 血管炎（IgAV），以前称为过敏性紫癜，是一种小血管血管炎，最常见于儿科患者，可影响许多内部器官，包括肾脏、肺部、胃肠道和中枢神经系统（CNS）。该病症的中枢神经系统表现包括高血压脑病、血栓形成、视神经病变、癫痫、中枢神经系统血管炎以及最近描述的一种称为后部可逆性脑病综合征（PRES）的现象。PRES 的症状包括高血压、精神状态改变和由血脑屏障的血管源性破坏引起的癫痫发作，通过磁共振成像在顶枕叶出现特征性与水肿相关的灰白质变化来诊断该病症。在此，我们报告了 IgAV 以 PRES 为首发表现的罕见病例，以提高对这种不常见关联的认识。
