Case Report
Central Nervous System Involvement in Henoch-Schonlein
Purpura in Children and Adolescents
Iliyana H. Pacheva,
1
Ivan S. Ivanov,
1
Krastina Stefanova,
1
Elena Chepisheva,
2
Lyubov Chochkova,
1
Dafina Grozeva,
1
Angelina Stoyanova,
1
Stojan Milenkov,
1
Penka Stefanova,
2
and Anna Petrova
3
1
Department of Pediatrics and Medical Genetics, Medical University-Plovdiv, Plovdiv, Bulgaria
2
Department of Pediatric Surgery, “St. George” University Hospital, Plovdiv, Bulgaria
3
Department of Imaging Diagnosis, “St. George” University Hospital, Medical University-Plovdiv, Plovdiv, Bulgaria
Correspondence should be addressed to Iliyana H. Pacheva; inapatcheva@hotmail.com
Received 8 July 2016; Revised 5 November 2016; Accepted 21 December 2016; Published 21 February 2017
Academic Editor: Abraham Gedalia
Copyright © 2017 Iliyana H. Pacheva et al. Tis is an open access article distributed under the Creative Commons Attribution
License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly
cited.
Central nervous system (CNS) involvement in Henoch-Schonlein purpura (HSP) is rare but poses diagnostic difculties. Te 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 fles of patients with HSP admitted at the Department of Pediatrics, Plovdiv, were studied retrospectively for a fve-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 afer 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 lef occipital lobe. Te patient experienced a second similar episode afer 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.
1. Introduction
Henoch-Schonlein purpura (HSP) is a systemic vasculitis
involving the small vessels. It occurs mainly in children;
over 75% of patients are under 10 years of age [1]. Its inci-
dence is 10–20 per 100,000 children [2–5]. Most com-
monly afected are the skin, joints, gastrointestinal tract, and
kidneys. CNS involvement in HSP is rare (0.65–8%) but
poses diagnostic difculties and sometimes has long-term
neurological sequelae [3, 6, 7]. Neurologic manifestations of
HSP were frst described by Osler [8] in 1914 as transitory
hemiparesis and decreased level of consciousness as a result
from either oedema or brain hemorrhage. Tere are few case
reports about the neurological manifestations of HSP (head-
ache, seizures, hemiparesis, aphasia, cortical blindness, and
impaired consciousness) [9–18] and even fewer studies [19,
20]. Many of them are written in the past century, when
MRI with new sequences was not readily available. Moreover
histologic confrmation of CNS involvement in HSP occurred
casuistically [21, 22]. Tat is why the pathogenic mechanisms
of CNS involvement in reported cases remain unclear. Con-
temporary studies on the characteristics of CNS involvement
in HSP with the use of modern imaging methods are needed
to extend our knowledge of this category.
2. Aim
Te aim is to establish the frequency of CNS involvement in
children with HSP and to analyze its clinical characteristics
and do a literature review.
Hindawi
Case Reports in Pediatrics
Volume 2017, Article ID 5483543, 6 pages
https://doi.org/10.1155/2017/5483543