STROKE AS A LIFE-THREATENING COMPLICATION IN CHILDREN WITH LINEAR SCLERODERMA OF FACE

Area of Science:

• Pediatric Neurology
• Dermatology
• Vascular Medicine

Background:

• Juvenile localized scleroderma (JLS) is a rare autoimmune condition affecting the skin and underlying tissues.
• Neurological complications in children with JLS, particularly those affecting the head and face, require further investigation.

Purpose of the Study:

• To determine the prevalence and spectrum of neurological disorders in children diagnosed with JLS.
• To identify risk factors for stroke in children with JLS affecting the head and face (LSH).

Main Methods:

• A cohort of 156 children with JLS underwent neurological examinations.
• Brain magnetic resonance imaging (MRI), electroencephalography (EEG), and genetic thrombophilia markers were assessed.
• Patients were categorized into two groups: those with LSH and those with JLS without facial plaques.

Main Results:

• Neurological disorders were present in 49% of children with LSH and 30% of those without facial plaques.
• Headaches, epileptic seizures, cranial nerve involvement, and stroke were observed in the LSH group.
• Brain MRI revealed abnormalities in 84% of children with neurological disorders, including white matter and gliotic lesions.
• Three cases of ischemic stroke were identified, all associated with genetic thrombophilia, vascular malformations, or antiphospholipid syndrome.

Conclusions:

• Sclerodermic vasculopathy, cerebral vascular anomalies, and genetic thrombophilia are identified as risk factors for stroke in children with LSH.
• Routine MRI angiography and genetic thrombophilia screening are recommended to identify high-risk children with LSH for stroke prevention.