Joint contractures and acroosteolysis in Ehlers-Danlos syndrome type IV

Among a series of 10 patients with Ehlers-Danlos syndrome Type IV, several had peripheral joint contractures and 3 had acroosteolysis. The consequent deformities simulated the appearance of chronic inflammatory joint disease and hence, patients with this rare syndrome may come to the attention of rheumatologists. Recognition of Ehlers-Danlos syndrome Type IV is important because of the risk of early death from arterial rupture. The syndrome is also of theoretical importance as an "experiment of nature" illustrating functional consequences of deficiency of Type III collagen.