Linear Scleroderma of Scalp 'en coup de sabre' in a Child Presenting with Rapidly Deteriorating Acute Encephalopathy

Background: Linear scleroderma (LS) is a variant of localized scleroderma, which is termed as “en coup de sabre” (ECDS) when it involves the head and/or face. Although mainly a dermatological condition, extracutaneous manifestations have been infrequently reported. Clinical Description: A 13-year-old boy presented with fever, altered sensorium with seizures, right hemiparesis, and right-sided upper motor neuron type of facial palsy. He was noticed to have a depression in the left frontoparietal area of the scalp with skin changes. Management and Outcome: The child was initially managed along the lines of acute encephalitis supported with mechanical ventilation. Brain imaging revealed tiny calcification in the left temporal and parietal lobes with altered signal intensities ipsilateral to the scalp lesion. Suspecting an association with the overlying scalp skin lesion, biopsy of the skin lesion was done which confirmed LS. The erythrocyte sedimentation rate was elevated and the antinuclear antibody was positive. The child was started on pulse methylprednisolone following which encephalopathy improved facilitating extubation. Later, he was started on subcutaneous methotrexate and he improved on follow-up. Conclusion: This case creates awareness regarding the association between neurological manifestations and LS EDCS, which responds favorably to immunosuppressives and methotrexate.