Arch Dis Child 98:19 doi:10.1136/archdischild-2012-302129
  • Miscellanea
  • Images in paediatrics

Linear scleroderma en coup de sabre and frontal lobe epilepsy

  1. Jeroen R Vermeulen2
  1. 1Department of Paediatrics, Meander Medical Centre, Amersfoort, The Netherlands
  2. 2Department of Paediatric Neurology, VU Medical Centre, Amsterdam, The Netherlands
  1. Correspondence to Dr Paul H G Hogeman, Department of Paediatrics, Meander Medical Centre, Postbox 1502, Amersfoort 3800 BM, The Netherlands; p.hogeman{at}
  • Received 16 June 2012
  • Accepted 2 October 2012
  • Published Online First 31 October 2012

A 5-year-old boy with 8 months' delay in speech development and 6 months' delay in fine motor skills presented with two episodes of paralysis of his arm without jerks or involuntary movements, followed by impairment of speech and loss of motor control over both legs, resolving within 15 min. Physical examination was normal except for joint hypermobility. No cerebral spinal fluid was obtained. MRI revealed subcortical T2 weighted hyperintensity in the left frontal white matter without contrast-enhancement and callosal cysts (figure 1B). During second examination a hypopigmented linear plaque was found on his left frontal scalp (figure 1A). EEG showed epileptic activity in the left frontal lobe. A combination of skin abnormality, ipsilateral cortical dysplasia and focal seizures led to the diagnosis of linear scleroderma (LS) or ‘en coup de sabre’ subtype, named after the distinct scars made by a sabre.

Figure 1

(A) Hypopigmented linear plaque on the left frontal scalp. (B) MRI with cortical dysplasia in the left frontal white matter.

LS is the most frequent subtype (65–67%) of localised scleroderma in children, consisting of thickening of the skin and subcutaneous tissue with collagen depositions.1 ,2 Face or head involvement are depicted as ‘en coup de sabre’. These patients can also show extracutaneous symptoms, such as neurological and ocular symptoms with prevalence near to 20%.1–3 Depending on location, size and depth of the plaques, therapy varies widely from expectative, Methotrexate to surgery. Prognosis depends on the depth, but mostly on the location. Plaques affecting joints can cause contractures and bone growth defects. This case highlights the importance of examining the scalp in paediatric patients with focal epileptic symptoms.


  • Competing interests None.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.