Presentation
Left frontoparietal linear band of alopecia and skin atrophy started 5 years ago with progressive course and current left nasal alar atrophy.
Patient Data
Left frontoparietal scalp linear band of atrophy with subjacent thinned calvaria.
Ipsilateral left high frontoparietal blurring of the gray-white matter interface.
Left frontoparietal scalp linear band of atrophy with subjacent thinned calvaria.
Ipsilateral left high frontoparietal blurring of the gray-white matter interface.
A photo of the patient's head showed a left frontoparietal linear band of alopecia and skin atrophy.
Annotated images demonstrate left frontoparietal scalp atrophy with subjacent calvarial thinning.
Case Discussion
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
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