Table 1

 The various types of Ehlers-Danlos syndrome

TypeInheritanceClinical features
AD, autosomal dominant; AR, autosomal recessive; XLR, X-linked recessive (adapted from Champion et al (page 2034).5
IADSoft, velvety, hyperextensible skin with easy bruising, cigarette paper scars, and pseudotumours
IIAD (Rarely AR)Milder but similar to type I
IIIADJoint involvement with early osteoarthritis and minimal skin abnormality
IVADIncreased susceptibility to great vessel and bowel rupture. Cutaneous features as in type I with normal joints.
VXLRSimilar to type II with more ecchymoses
VIARCutaneous features as in type I but with ocular involvement such as keratoconus and ocular fragility.
VII A + BADProminent musculoskeletal features with floppy infant, congenital hip dislocation, and short stature. Normal scarring.
VII CARPronounced hypermobile joints with very soft, fragile skin and easy bruising.
VIIIADSimilar to type II with severe periodontitis
IXXLRHyperextensible skin with short arms, occipital horns, broad clavicles, and bladder diverticula.
XARSimilar to type II