gms | German Medical Science

102. Jahrestagung der DOG

Deutsche Ophthalmologische Gesellschaft e. V.

23. bis 26.09.2004, Berlin

A rare ophthalmologic manifestation in a patient with scleroderma “en coup de sabre”

Meeting Abstract

  • corresponding author J. Klink - University Eye Hospital, Würzburg
  • H. J. Girschick - University Children`s Hospital, Würzburg
  • T. Klink - University Eye Hospital, Würzburg
  • H. Steffen - University Eye Hospital, Würzburg
  • W. Schrader - University Eye Hospital, Würzburg

Evidenzbasierte Medizin - Anspruch und Wirklichkeit. 102. Jahrestagung der Deutschen Ophthalmologischen Gesellschaft. Berlin, 23.-26.09.2004. Düsseldorf, Köln: German Medical Science; 2004. Doc04dogP 113

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Veröffentlicht: 22. September 2004

© 2004 Klink et al.
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Linear scleroderma "en coup de sabre" is a rare form of a progressive hemifacial atrophy possibly associated with neurological and ophthalmological complications, the pathogenesis of is still unclear. We report on a case of a rare retinal association in a patient with scleroderma circumscripta.

Case report

A 10 year old boy with a distinct skin- and bone atrophy involving left forehead and orbital rim diagnosed as Scleroderma circumscripta "en coup de sabre" in 1998 was presented to exclude an ophthalmological involvement. The biomicroscopy of the left fundus showed a saccular venous dilatation at the inferonasal vascular arcade. Initially it has been diagnosed as venous malformation or even retinal cavernous hemangioma. Furthermore there was an anisocoria of 3 mm (left>right) without an afferent pupillary defect. The anterior segment and extraocular muscles were not involved. 1.5 years later a marked subretinal exudation within the retinal venous vascular changes occurred. Other retinal vessels and the fellow eye remained normal. Prior to the exudation cranial MRI showed inflammatory changes which were further diagnosed as cerebral vasculitis. A further progression could be avoided so far by immunosuppressive therapy with Mycophenolat mofetil since 2 months.


Previous published reports on ophthalmological manifestation mainly describe orbital and extraocular muscle involvement. There is just one case report on an ipsilateral Coat`s reaction in the eye of a child with en coup de sabre morphea (Neki et al, Indian J Ophthalmol 1992) which is similar to our patient. Regular fundus examination in patients with scleroderma (e.g. with cerebral inflammatory manifestation) are recommended.