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
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Left frontoparietal scalp linear band of atrophy with subjacent thinned calvaria.
Ipsilateral left high frontoparietal blurring of the gray-white matter interface.
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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.
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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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