Table 1.

The Villefranche Criteria for Major Ehlers-Danlos Syndrome Subtypes

Subtype	Major Criteria	Minor Criteria
Classic	Skin extensibility Widened atrophic scars Joint hypermobility	Smooth, velvety skin Molluscoid pseudotumors Subcutaneous spheroids Complication of joint hypermobility Muscle hypotonia, motor delay Easy bruising Manifestation of tissue extensibility Surgical complication Positive family history
Hypermobility	Hyperextensible and/or smooth, velvety skin Generalized joint hypermobility	Recurring joint dislocations Chronic joint/limb pain Positive family history
Vascular	Thin, translucent skin Arterial/intestinal/uterine fragility or rupture Extensive bruising Characteristic facial appearance	Acrogeria Hypermobility of small joints Tendon and muscle rupture Talipes equinovarus Early-onset varicose veins Arteriovenous, carotid-cavernous, carotid-cavernous sinus fistula Pneumothorax/pneumohemothorax Gingival recessions Positive family history, sudden death in a close relative
Kyphoscoliotic	Generalized joint hypermobility Congenital hypotonia Congenital and progressive scoliosis Scleral fragility and rupture of the ocular globe	Tissue fragility, including atrophic scars Easy bruising Arterial rupture Marfanoid habitus Microcornea Osteopenia/porosis Positive family history
Arthrochalasis	Generalized joint hypermobility and recurrent subluxations Congenital bilateral hip dislocation	Skin hyperextensibility Tissue fragility, including atrophic scars Easy bruising Hypotonia Kyphoscoliosis Osteopenia/porosis
Dermatosparaxis	Severe skin fragility Sagging, redundant skin	Soft, doughy skin texture Easy bruising Premature rupture of fetal membranes Large hernias (umbilical, inguinal)