IPSILATERAL INFLAMMATORY WHITE MATTER LESIONS WITH HEMORRHAGIC FOCI IN LINEAR SCLERODERMA EN COUP DE SABRE: BRAIN MRI FINDINGS WITHIN THE PARRY–ROMBERG SPECTRUM

Linear scleroderma en coup de sabre is a rare subtype of localized scleroderma that overlaps with Parry–Romberg syndrome. We report the case of a 35-year-old woman with long-standing linear scleroderma en coup de sabre who underwent brain MRI to evaluate possible intracranial involvement despite the absence of neurological symptoms.
MRI revealed ipsilateral white matter hyperintensities with thin peripheral contrast enhancement, suggestive of active inflammatory changes, associated with deep hemorrhagic foci on T2*-weighted imaging involving the left internal capsule and thalamic region. These findings were consistent with subclinical intracranial involvement within the Parry–Romberg spectrum.
This case emphasizes the importance of brain MRI in detecting and characterizing inflammatory and hemorrhagic brain abnormalities in patients with craniofacial linear scleroderma, even in the absence of neurological symptoms.