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Pediatric Rheumatology logoLink to Pediatric Rheumatology
. 2014 Sep 17;12(Suppl 1):P358. doi: 10.1186/1546-0096-12-S1-P358

A case of Henoch-Schönlein purpura associated with posterior reversible encephalopathy and review of litereature

Kyu Yeun Kim 1,, Mo Kyung Jung 1, Ki Hwan Kim 2, Dong Soo Kim 2
PMCID: PMC4191715

Introduction

Henoch-Schönlein purpura (HSP) is a vasculitis that involves small vessels and seen in children predominantly. Main symptoms are purpuric rashes on body, especially on lower extremities, arthralgia, abdominal pain, and nephritis. Uncommonly, nervous system can be involved.

Objectives

PRES can occur in HSP infrequently. Here we will review our case, a first reported Korean case, and compare with published cases to find out which symptoms and signs should be aware.

Methods

First, our case is reviewed retrospectively by electrical medical record. And then, we searched Pubmed database, terms including HSP, PRES, RPLS, and encephalopathy. We compared collected cases and our case.

Results

A 8-year-old girl visited our hospital complaining abdominal pain and purpuric rash on lower extremities and buttock. On hospital day 7, there were two brief events of generalized tonic-clonic seizure. She complained dizziness and blurred vision. Brain MRI demonstrated increased signal intensity in the cortex and subcortical white matter, in the parietooccipital area, and impression was PRES. She was discharged on day 19 without any complication. Table 1.

Table 1.

Age Sex Main symptom Neurologic symptom Blood pressure Thrapy
Our case 8yr F Abdominal pain, Blood tinged stool, Purpura GTC, Blurred vision 144/86 MPT pulse Tx, anti-hypertensive drug, anti-convulsive drug

Dasarathi, et al. 11yr F Abdominal pain, vomiting, purpura GTC, complete loss of vision 112/67 supportive Tx

Sivrioglu, et al. 5yr F purpura, arthralgia, abdominal pain headache seizure 180/110 anti-hypertensive drug, anti-convulsive drug, dialysis

Sasayama, et al. 13yr F abdominal pain, purpura generalized seizure, cortical blindness 180/120 MTP pulse Tx, anti-hypertensive drug

Fuchigami, et al. 7yr F abdominal pain, arthralgia, purpura sudden loss of vision GTC 190/100 PL, anti-hypertensive drug, anti-convulsive drug

Woolfenden et al. 10yr M fever, RLQ pain, bloody diarrhea Bi-temporal headache, nausea, vomiting bilateral visual loss seizure not known MTP pulse Tx, anti-convulsion drug

Ozcakar et al. 10yr M fever, palpable purpra, arthralgia seizure 130/90 steroid, anti-convulsive Tx, anti-hypertension drug

Endo et al. 7yr F abdominal pain, purpura seizure unconsciousness loss of vision 190/100 PL, anti-hypertensive drug, anti-convulsive drug

Conclusion

In HSP patients, hemodynamic change due to severe hypertension and renal insufficiency, and CNS vasculitis can cause PRES. JI Shin suggests IL-6 and VEGF can play a role in this situation. Neurological involvement is not common in HSP. But when patients complain headache or blurred vision, it can be manifestation of CNS lesion, including PRES. Adequate assessment and manage should be performed to avoid neurologic sequela.

Disclosure of interest

None declared


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