Table 1.

Clinical Features of classical and periodontal Ehlers-Danlos syndrome (EDS) in the present family.

	Proband IV:2	Mother III:2	Grandfather II:2
Major criteria of classical EDS
Joint hypermobility	+	+	+
Hyperelastic and soft skin	+	+	−
Atrophic scars	+	+	n.a.
Major criteria of periodontal EDS
Early severe periodontitis	+	−	−
Lack of attached gingiva	+	−	n.a.
Pretibial hyperpigmentation	+	−	−
Minor criteria of classical and / or periodontal EDS
Acrogeric face and hands	−	−	−
Myopia	−	+	−
Blue sclerae	+	−
Ectopia lentis	−	−	−
epicanthic folds	−	+
Arachnodactyly	−	−	−
multiple subcutaneous papules	+	+
Easy bruising	+	+	+
Poor wound healing	+	+	+
dilatation of aorta	−	−	n.a.
Hernias	−	umbilical hernia	−
Organ rupture	−	−	−
Recurrent infections	+	+	+
Involvement of heart valves	hypermobile mitral valve (no prolapse)	n.a.	n.a.
Luxation of knee caps	+	+