Monday, December 10, 2012

Linear Scleroderma en Coup de Sabre

Linear scleroderma is a condition characterized by localized linear atrophy that resembles the findings one would find, at the end of the Franco-Prussian War, in soldiers who had been injured from cavalry charges from the saber-wielding Prussian Cavalry.  Since this description is in French and not in German, one would correctly presume the French bore the brunt of these kinds of injuries.

The onset of this condition is, as above, in childhood and usually is absent of underlying neurological deficits, unlike its cousin Parry Romberg Syndrome, which is a more severe condition involving progressive facial hemiatrophy, which can result in the entire affected side of the face being smaller than the other.  On a clinical basis, without this difference from side to side, one would have to lean on en Coup de Sabre being the preferred diagnosis.  This is just such a case.  

Linear Scleroderma en Coup de Sabre is usually unaccompanied by routine laboratory abnormalities, except in research settings.  Thus, although Scl-70 and ANA titers can be out of whack, usually they are not.  In fact, some authors question whether this entity is at all related to morphea or scleroderma.  

This disease does not, in its pure form, extend below the orbital ridge, although there are overlapping cases with Parry Romberg which go lower.  If you study the case above, you can see the atrophy, the superficial nature of the vasculature and the linear nature of the defect.

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