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
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