Congenital contractural arachnodactyly
Congenital contractural arachnodactyly (CCA; MIM 121050) is an autosomal dominantly inherited connective tissue disorder characterized by contractures of peripheral joints, and arachnodacdyly. Findings such as kyphoscoliosis, crumbled ear helices, high-arched palate and mitral valve prolapse are also common. Even though patients with CCA may have marfanoid habitus, they do not exhibit ectopia lentis. CCA is caused by mutations in FBN2. This gene codes for fibrillin 2, a component of microfibrils.