Linear scleroderma


Diagnosis confirmed by skin biopsy.

Linear scleroderma is a rare focal form of scleroderma. It occurs in both children and adults. It's classically characterized by a linear band of atrophy involving the frontal or frontoparietal scalp and subjacent thinned calvaria associated with ipsilateral focal brain abnormalities.

Linear scleroderma may coexist with progressive facial hemiatrophy (Parry-Romberg syndrome).

Radiological features in the brain beneath the skin lesion include focal atrophy, blurring of the gray-white matter interface, calcifications, and cerebral microhemorrhages