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Extracranial linear localized scleroderma associated with longstanding epileptic encephalopathy unresponding to methotrexate and prednisone

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We report a boy, who achieved developmental milestones without delay, presenting complex partial seizures (left-deviation of the head, staring gaze, oral automatism and tonic-clonic generalization) at 7 years: his blood/cerebrospinal fluid tests were all within normal limits, enclosed those for inborn errors of metabolism. Progressive signs of psycho-motor regression were simultaneously observed by caregivers in combination with a band of linear ivory-coloured indurated lesion on the dorsal region of the right foot, which was diagnosed as linear localized scleroderma (LS). Immunological tests performed at 8 years revealed only low-titre positivity of anti-nuclear antibodies. Various brain CT and MRI scans resulted negative. In the following years tonic-clonic seizures were observed with daily recurrence in spite of different antiepileptic drugs. At 16 years we evaluated this patient for the first time: asymmetrical tonic fits had multiple daily frequency and therapy consisted of clobazam/methsuximide; EEG revealed dysrupted electrical cerebral activity; brain MRI was normal; the skin lesion was extended to the whole right inferior limb. Methotrexate (MTX) and prednisone were instituted (respectively at the dosage of 15 mg/m2/week and 1.5 mg/kg/day): MTX was maintained for 1,5 year with low-dose prednisone (0.5 mg/kg/day). Semeiological features of seizures remained unchanged and no control upon their frequency could be reached, whilst LS remained stable.

The exact nature of multi-resistant epilepsy associated with extra-cranial linear LS remains a matter of debate and the immunologic process leading to the pathologic collagen deposition might be dissociated from the specific neurological disturbance.

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Correspondence to D Rigante.

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Open Access This article is published under license to BioMed Central Ltd. This is an Open Access article is distributed under the terms of the Creative Commons Attribution 2.0 International License (, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Rigante, D., Battaglia, D., Contaldo, I. et al. Extracranial linear localized scleroderma associated with longstanding epileptic encephalopathy unresponding to methotrexate and prednisone. Pediatr Rheumatol 6, P227 (2008) doi:10.1186/1546-0096-6-S1-P227

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  • Methotrexate
  • Prednisone
  • Developmental Milestone
  • Complex Partial Seizure
  • Fluid Test