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| Ehlers-Danlos Syndrome IV (vascular type) Diagnostic Criteria |
The EBMcalc module Ehlers-Danlos Syndrome IV (vascular type) Diagnostic Criteria is available in EBMcalc Complete Edition.
This calculator evaluates the following Clinical Criteria:
- Thin, translucent skin, especially on the chest or abdomen
- Arterial/intestinal/uterine fragility or rupture
- Easy bruisability
- Characteristic facial appearance of thin lips and philtrum, small chin, thin nose, large eyes
- Acrogeria (an aged appearance to the extremities / hands)
- Hypermobility of small joints
- Tendon/muscle rupture
- Early-onset varicose veins
- Arteriovenous carotid-cavernous sinus fistula
- Pneumothorax/pneumohemothorax
- Chronic joint subluxations/dislocations
- Congenital hip dislocation
- Club foot (talipes equinovarus)
- Gingival recession
- A family history consistent with autosomal dominant inheritance
References
- Beighton P, De Paepe A, Steinmann B, Tsipouras P, Wenstrup RJ. Ehlers-Danlos syndromes: revised nosology, Villefranche, 1997. Ehlers- Danlos National Foundation (USA) and Ehlers-Danlos Support Group (UK). Am J Med Genet. 77:31-7. PubMed ID: 9557891
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