Although the exact role of CAH-related hormonal factors and/or chronic glucocorticoid treatment in connective tissue dysplasia is not yet completely understood, it has been shown that CAH-X patients are consistently more severely affected than patients with homozygous or heterozygous TNX-deficient-type EDS without CAH (11, 12, 16, 19, 25). This evidence concerns the gene TNXB and congenital adrenal hyperplasia.