Severe epilepsy preceding by four months the onset of scleroderma en coup de sabre

Juvenile localized scleroderma (JLS) includes several subtypes including plaque morphea, linear scleroderma and the en coup de sabre type which affects face and head. The latter variety may involve the eye and the brain with various appearance and clinical complications.We describe the case of a 6-y...

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Bibliographic Details
Published in:Clinical and experimental rheumatology 2009-05, Vol.27 (3 Suppl 54), p.64-67
Main Authors: Sartori, S, Martini, G, Calderone, M, Patrizi, A, Gobbi, G, Zulian, F
Format: Article
Language:English
Subjects:
Brain - pathology
Child
Disease Progression
Epilepsy - diagnosis
Epilepsy - drug therapy
Face
Humans
Magnetic Resonance Imaging
Male
Scleroderma, Localized - diagnosis
Scleroderma, Localized - pathology
Skin - pathology
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Summary:Juvenile localized scleroderma (JLS) includes several subtypes including plaque morphea, linear scleroderma and the en coup de sabre type which affects face and head. The latter variety may involve the eye and the brain with various appearance and clinical complications.We describe the case of a 6-year-old boy who presented partial complex seizures, with status epilepticus, four months before the appearance of sclerodermatous skin lesions on the face. This case report raises important questions on the pathogenesis of JLS and, particularly, on the issue whether it is a mere autoimmune condition or a neuro-cutaneous disease.
ISSN:0392-856X