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. 2017 Sep 15;56(20):2791–2796. doi: 10.2169/internalmedicine.8758-16

Table 2.

Current Classification of Ehlers-Danlos Syndromes.

Descriptive Clinical features Genes Inheritance
Classical Marked joint hypermobility, skin hyperextensibility, bruising, abnormal scarring COL5A1, COL5A2 AD
Hypermobility Marked joint hypermobility, minor skin findings Largely unknown, TNXB AD
Vascular Thin translucent skin, marked bruising, small joint hypermobility, high risk for rupture of arteries, bowel and gravid uterus COL3A1 AD
Kyphoscoliosis Kyphoscoliosis recalcitrant to surgery, joint hypermobility, risk for arterial rupture, ocular fragility PLOD1 AR
Arthrochalasia Marked joint hypermobility, bilateral congenital hip dislocation COL1A1, COL1A2 AD
Dermatosparaxis Soft, fragile skin with late onset skin redundancy, blue sclerae, joint hypermobility ADAMTS2 AR

AD: autosomal dominant fashion, AR: autosomal recessive fashion