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Central Nervous System Involvement in Henoch-Schonlein Purpura in Children and Adolescents
Iliyana H Pacheva1, Ivan S Ivanov1, Krastina Stefanova1
1Department of Pediatrics and Medical Genetics, Medical University-Plovdiv, Plovdiv, Bulgaria.
Insights
Central nervous system involvement in Henoch-Schonlein purpura (HSP) is rare. This study found one case of Posterior Reversible Encephalopathy Syndrome (PRES) in 112 children with HSP, highlighting the need for clinical awareness.
Area of Science:
- Pediatrics
- Neurology
- Rheumatology
Background:
- Henoch-Schonlein purpura (HSP) is a common childhood vasculitis.
- Central nervous system (CNS) involvement in HSP is infrequent but challenging to diagnose.
- Posterior Reversible Encephalopathy Syndrome (PRES) is a rare neurological complication.
Observation:
- A retrospective study analyzed 112 children diagnosed with HSP between 2009-2013.
- One 8-year-old girl presented with atypical HSP, including abdominal pain, rash, hypertension, and neurological symptoms.
- Neurological manifestations included visual disturbances, seizures, hemiparesis, and confusion, consistent with PRES.
Findings:
- Central nervous system involvement occurred in 0.9% of pediatric HSP cases.
- The CNS involvement presented as Posterior Reversible Encephalopathy Syndrome (PRES), potentially linked to vasculitis or hypertension.
- Neuroimaging revealed characteristic occipital lobe lesions, which resolved completely with clinical improvement.
Implications:
- HSP should be considered as a potential cause of PRES in pediatric patients.
- Early recognition and management of PRES in HSP are crucial to prevent diagnostic and therapeutic delays.
- Increased clinical awareness of this rare complication can improve patient outcomes.
Abstract:
Central nervous system (CNS) involvement in Henoch-Schonlein purpura (HSP) is rare but poses diagnostic difficulties. The aim of the study was to establish the frequency of CNS involvement in HSP, to analyze its clinical characteristics and do a literature review. Medical files of patients with HSP admitted at the Department of Pediatrics, Plovdiv, were studied retrospectively for a five-year period (2009-2013). Diagnosis was based on the American College of Rheumatology criteria. Out of 112 children with HSP 1 case (0.9%) had CNS involvement presenting as Posterior Reversible Encephalopathy Syndrome (PRES), which may be a result of CNS vasculitis or arterial hypertension. It was an 8-year-old girl with atypical HSP which started with abdominal pain requiring surgery. On the third day after the operation a transient macular rash and arterial hypertension appeared, followed by visual disturbances, hemiconvulsive epileptic seizures, postictal hemiparesis, and confusion. Head CT showed occipital hypodense lesions and MRT-T2 hyperintense lesion in the left occipital lobe. The patient experienced a second similar episode after 2 weeks when palpable purpura had also appeared. Neurological symptoms and MRI resolved completely. HSP can be an etiological factor for PRES in childhood. Although PRES is a rare complication of HSP, clinicians must be aware of it and avoid diagnostic and therapeutic delays.
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