[Ehlers-Danlos type IV syndrome presenting as renovascular hypertension]

The association of Ehlers-Danlos type IV syndrome and acrogeria with renovascular hypertension is reported. Ultrastructural abnormalities observed in our case were different of those associated with acrogeria. We propose that acrogeria associated with Ehlers-Danlos type IV syndrome be a different disease of acrogeria of Gottron. The occurrence of renovascular hypertension in Ehlers-Danlos is unusual.