Definition of Syndrome, Danlos
Syndrome, Danlos: Better known today as Ehlers-Danlos syndrome (EDS), is an inherited disorder with easy bruising, joint hypermobility (loose joints), skin laxity, and weakness of tissues. There are a number of different Ehlers-Danlos syndromes which share these features but can be categorized into nine different types.
- Type I EDS (the "gravis" form of EDS) is characterized by marked joint hypermobility, skin hyperextensibility (laxity), and fragility. Joint dislocations and scoliosis. It is inherited as an autosomal (non-sex-linked) dominant genetic trait. Dominant means that a single gene is capable of producing the disease.
- Type II EDS (the "mitis" form of EDS) is similar to type I, but is less severe. It, too, is inherited as an autosomal dominant genetic trait.
- Type III EDS (the benign hypermobility form of EDS). Joint hypermobility is the major manifestation. It is inherited as an autosomal dominant genetic trait.
- Type IV EDS (the arterial form) Spontaneous rupture of arteries and bowel is a serious manifestation. Skin laxity is variable. It is inherited as an autosomal dominant and recessive genetic trait.
- Type V EDS is clinically similar to type II but is X- linked. The gene is on the X chromosome). If a woman is carrying the gene, the chance for each of her children her son to receive the gene is 50:50. A son with the gene is affected with the disease while a daughter with the gene is merely a carrier like her mother.
- Type VI EDS (the ocular-scoliotic form of EDS) is characterized by a fragile globe of the eyes, significant skin and joint laxity, and severe curvature of the spine (scoliosis). It is inherited as an autosomal (non-sex- linked) recessive genetic trait. Recessive means that two copies of the gene are required to produce the disease.
- Type VII EDS (arthrochalasis multiplex congenita). Patients with this type of EDS are short in height and severely affected by joint laxity and dislocations. Skin involvement is variable. Autosomal dominant and recessive inheritance is possible.
- Type VIII EDS. Patients have different degrees of joint hypermobility and inflammation of the gums and bone adjacent to the teeth (periodontitis).
- Type IX EDS. Patients have mildly hypermobile joints and can have mitral valve prolapse. It is inherited as autosomal dominant.
Last Editorial Review: 6/14/2012
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