The classical type of Ehlers-Danlos Syndrome may vary in severity from mild to severe.


Former terms (Berlin, 1988): EDS Type I (gravis) and EDS Type II (mitis)


Inheritance: autosomal dominant


Major criteria (one or more needed for diagnosis):

(1) skin hyperextensibility

(2) widened atrophic scars

(3) joint hypermobility


Minor criteria (supportive but insufficient for diagnosis without a major criterion):

(1) smooth, velvety skin

(2) molluscoid pseudotumors (fleshy lesions associated with scars, commonly found over pressure points)

(3) subcutaneous spheroids (subcutaneous spherical hard bodies, frequently mobile and palpable on the forearms and shins, may become calcified)

(4) joint complications arising from hypermobility, including recurrent joint subluxations.

(5) muscle hypotonia and/or delayed gross motor development

(6) easy bruisability

(7) poor wound healing with postoperative hernias and other surgical complications

(8) complications of tissue hyperextensibility and fragility (hiatal hernia, anal prolapse, varicose veins, etc.)

(9) prematurity of affected newborns


Criteria shared with other forms of EDS:

(1) mitral valve prolapse may occur

(2) dyspareunia and sexual dysfunction

(3) fatigue


Biochemical findings: some families show a defect in type V collagen, but no single test has proved reliable for diagnosis of the classical type.


To read more or access our algorithms and calculators, please log in or register.