March 5, 2019
A 9-year-old girl referred for a routine brain MRI by the neurologist, in the setting of her primary illness.
LINEAR SCLERODERMA EN COUP DE SABRE
- Background
- Linear scleroderma is characterized by sclerotic lesions distributed in a linear, band-like pattern in the frontoparietal area of the head and is a unique form of localized scleroderma that primarily affects the pediatric population, with 67% of patients diagnosed prior to 18 years of age.
- En coup de sabre and Parry–Romberg syndrome (progressive hemifacial atrophy), are variants of linear morphea on the head and neck that can be associated with neurologic manifestations.
- The range of neurologic symptoms include seizures, recent onset headaches, focal neurologic deficits and movement disorders, behavioral changes, or progressive intellectual deterioration.
- Imaging
- Typically ipsilateral to the skin lesions in the cerebral hemisphere.
- Intraparenchymal calcifications can be found in the basal ganglia and subcortical white matter.
- White matter hyperintensities typically involve the subcortical white matter.
- Parenchymal and leptomeningeal enhancement may also be seen.
- Cerebral atrophy is generally focal and subtle.
- Diagnosis
- The diagnosis of linear scleroderma is made based on the clinical characteristics of the cutaneous and soft tissue findings.
- Treatment
- Immunosuppressive agents might be considered to stop the progressive atrophy of the brain and controlling seizures.
