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CLASSICAL TYPE (Formerly EDS Types I & II)
Marked skin hyperextensibility (stretchy) with widened atrophic scars and
joint hypermobiliity are found in the Classical Type of EDS. The skin manifestations
range in severity from mild to severe expression. The skin is smooth and velvety
with the evidence of tissue fragility and easy bruisability. Examples of tissue
extensibility and fragility include hiatal hernia, anal prolapse in childhood
and cervical insufficiency. Hernias may be a post-operative complication. Scars
are found mostly over pressure points such as the knees, elbows, forehead and
chin. Calcified hematomas associated with scars are frequently found over pressure
points such as the elbows, and fat containing cysts are usually found on the forearms
and shins.
Complications of joint hypermobility include sprains, dislocations/subluxations
and flatfoot to name a few. Recurrent joint subluxations are common in the shoulder,
patella and temporomandibular joints. Muscle hypotonia and delayed gross motor
development may also be evident.
Clinical Testing -- Abnormal electrophoretic mobility of the Procx1(V) or procx2(V)
chains of collagen type V has been detected in several but not all families with
the Classical Type. The Classical Type of EDS is inherited in an autosomal dominant
manner.
Types of EDS Main Page
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