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Table 1 Major, minor, and minimal criteria according to the 2017 nosology of classical Ehlers-Danlos syndrome

From: Multisystemic manifestations in a cohort of 75 classical Ehlers-Danlos syndrome patients: natural history and nosological perspectives

  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
  1. Abbreviations: N number of patients presenting the investigated feature, T total number of patients in whom the feature was investigated
  2. Significant p-values < 0.05 are in bold
  3. A: p-values females vs males
  4. B: p-values patients < 18 years vs patients ≥18 years