Bett er known today as Ehlers-Danlos syndrome (EDS),
this 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 od 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.
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