Iowa Branch of the Ehlers-Danlos National Foundation: Information and Support for Iowa Families with Ehlers-Danlos Syndrome
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Types of EDS

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.

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