Three patients with linear scleroderma en coup de sabre who presented with neurologic abnormalities before or concurrent with the dermatologic diagnosis are reported from the Medical College of Wisconsin, Milwaukee, WI. Case 1, a 5-year-old girl presented with partial complex seizures and a linear plaque with alopecia from the left eyebrow to the scalp. The EEG showed focal epileptiform discharges in the left posterotemporal area. Despite treatment with topical corticosteroids, the skin lesions first spread to form indurated plaques, and stabilized after 18 months. Seizures responded to antiepileptic drugs (AED), and treatment was tapered after 4 years. The child was seizure-free for 8 years, and AED were restarted at age 14, after a generalized seizure recurrence. Repeat MRIs were normal. Skin lesions consisted of hyperpigmentation and linear depressed plaques over the forehead. Case 2, a 6-year-old boy presented with right facial palsy, and headaches. Annular, erythematous areas of alopecia appeared on the right parietal scalp several months later. MRI was normal. Neurologic and skin lesions stabilized after treatment with prednisolone and methotrexate. Case 3, a 5 year-old girl presented with a white patch in the midline of her forehead, and 2 months later, she had a seizure. Uncontrolled partial complex seizures followed. Video-EEG showed a left frontal-temporal focus, a repeat MRI showed a T2 signal in the left subcortical white matter, CT revealed frontal calcifications and encephalomalacia, and PET scan had hypometabolic areas in the left frontal lobe. Resection of the left frontal area showed lymphocytic inflammation and focal vasculitis, secondary to linear scleroderma en coup de sabre. She was treated with methotrexate, and had no recurrence of seizures. [1]

COMMENT. Examination of the skin and scalp is important in children with unexplained partial complex seizures or other neurologic findings. A list of neurologic abnormalities associated with linear scleroderma en coup de sabre includes hemiparesis, peripheral facial palsy, oculomotor nerve palsy, ptosis, tongue atrophy, trigeminal neuralgia, intracranial aneurysm, subdural hygroma, and headaches. Neurologic complications usually follow the skin lesions by months or sometimes, years. The etiology is usually unknown, but the vasculitis has been attributed to infection (Borrelia burgdorferi in Japan and Europe), trauma, and genetic factors. Linear scleroderma is a self-limited disease, but reactivation can occur, and complete resolution is uncommon.