FATAL HEMOPTYSIS IN EHLERS-DANLOS SYNDROME - OLD MALADY WITH A NEW CURSE

We describe the case of a 27-year-old man with Ehlers-Danlos syndrome, type IV, The patient had recurrent and eventually fatal pulmonary hem orrhage. Type IV Ehlers-Danlos syndrome is a rare disorder of type III collagen synthesis. It is characterized by an unusual facies, thin, t ranslucent skin with venous vascular pattern, and hypermobility of the small joints. The cause of death is usually due to rupture of a viscu s or a major arterial hemorrhage and, in women, rupture of the gravid uterus, Fatal lung hemorrhage in Ehlers-Danlos syndrome, to our knowle dge, has not been previously described.