Table 1.

Major, minor, and minimal criteria according to the 2017 nosology of classical Ehlers-Danlos syndrome

	Total		Males		Females		p-valueA	Patients < 18		Patients ≥ 18		p-valueB
	N/T	%	N/T	%	N/T	%		N/T	%	N/T	%
Major Criteria
Generalized skin hyperextensibility plus extensive atrophic scarring	59/75	78.67	26/30	86.66	33/45	73.33	.274	24/31	77.41	35/44	79.54	.948
Generalized joint hypermobility (BS ≥ 5)	44/75	58.66	14/30	46.66	30/45	66.66	.138	19/31	61.29	25/44	56.81	.881
Minor Criteria
Easy bruising	65/75	86.66	25/30	83.33	40/45	88.88	.728	29/31	93.54	36/44	81.81	.181
Soft, doughy skin	59/75	78.66	24/30	80.00	35/45	77.77	.954	24/31	77.41	35/44	79.54	.948
Skin fragility (or traumatic splitting)	67/75	89.33	28/30	93.33	39/45	86.66	.593	30/31	96.77	37/44	84.09	.129
Molluscoid pseudotumors	22/75	29.33	13/30	43.33	9/45	20.00	.055	10/31	32.25	12/44	27.27	.834
Subcutaneous spheroids	18/65	27.60	8/24	33.33	10/41	24.39	.623	10/29	34.48	8/36	22.22	.412
Inguinal/umbilical/incisional hernia	27/75	36.00	10/30	33.33	17/45	37.77	.883	18/31	58.06	9/44	20.45	.002
Epicanthal folds	14/75	18.66	6/30	20.00	8/45	17.77	.951	10/31	32.25	4/44	9.09	.025
Complications of joint hypermobility (at least 1)	70/75	93.33	28/30	93.33	42/45	93.33	.636	28/31	90.32	42/44	95.45	.683
Bilateral pes planus	62/75	82.66	25/30	83.33	37/45	82.22	.851	27/31	87.09	35/44	79.54	.588
Sprains	34/75	45.33	17/30	56.66	17/45	37.77	.169	8/31	25.80	26/44	59.09	.008
Pain	30/75	40.00	12/30	40.00	18/45	40.00	.809	3/31	9.67	27.44	61.36	.001
Subdislocations	22/75	29.33	8/30	26.66	14/45	31.11	.876	6/31	19.35	16/44	36.36	.181
Dislocations	16/75	21.33	9/30	30.00	7/45	15.55	.226	1/31	3.22	15/44	34.09	.001
Two or more of these complications	41/70	58.57	19/28	67.85	22/42	52.38	.298	10/28	35.71	31/42	73.80	.003
Family history of a first degree relative who meets clinical criteria	42/75	56.00	17/30	56.66	25/45	55.55	.886	10/31	32.25	32/44	72.72	.001
Minimal Criteria	59/75	78.66	26/30	86.66	33/45	73.33	.274	24/31	77.41	35/44	79.54	.948
Major criterion 1 plus major criterion 2	37/75	49.33	13/30	43.33	24/45	53.33	.540	14/24	58.33	23/44	52.27	.822
Major criterion 1 plus at least 3 minor criteria	22/75	29.33	13/30	43.33	9/45	20.00	.055	10/24	41.566	12/44	27.27	.963

Abbreviations: N number of patients presenting the investigated feature, T total number of patients in whom the feature was investigated

Significant p-values < 0.05 are in bold

A: p-values females vs males

B: p-values patients < 18 years vs patients ≥18 years