A case of pediatric cerebral infarction as an intracranial manifestation of possible scleroderma en coup de sabre

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  • 脳梗塞の後に皮膚症状が出現し，剣創状強皮症と診断した小児脳梗塞の1例

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Abstract

<p>The patient was a 13-year-old woman. At five years of age, she had a cerebral infarction with right-sided motor and sensory disturbance. She was diagnosed with cerebral infarction with unknown etiology and treated with oral aspirin. Aspirin was discontinued after 3 years, and the patient has had no recurrence since then. At 12, she developed sudden onset of right upper and lower limb paralysis, and her head MRI showed cerebral infarction lesions in the left basal ganglia. Various investigations revealed no cardiac disease, thrombogenicity, or vascular abnormality. She was referred to our hospital for further investigation. The patient presented with a left forehead to the parietal skin lesion with skin laceration and thinning and atrophy of adipose tissue in the same region, which was consistent with scleroderma en coup de sabre. Scleroderma en coup de sabre is focal scleroderma that occurs on one side of the face to the frontal region and is sometimes associated with intracranial lesions. Our case was unique in that there was no evidence of skin lesions at the initial onset, and skin symptoms appeared afterward.</p>

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