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Cliedocranial Dysotosis
Cliedocranial Dysotosis
of fontanelles Delayed loss of the primary decidous: Delayed appearance of permanent teeth; Unusual shaped teeth; Supernumerary teeth Ehlors Danlos Type 4 Is an autosomal dominant defect in the type-III collagen synthesis; affecting approximately 1 in 100,000 to 250,000 people. Type 4 (vascular type) is considered one of the more serious forms of EhlersDanlos syndrome because blood vessels and organs are more prone to tearing (rupture). Patients with EDS type 4 express a characteristic facial appearance: large eyes, small chin, thin nose and lips, lobeless ears, have a small stature with a slim build, and typically have thin, pale, translucent skin (veins can usually be seen on the chest and abdomen) with very easy bruising and propensity to develop ecchymoses.