Table 1.
Comparison of the clinical phenotypes of the 24 EV patients described in this study
PatientCaseSexAge (yr)Age onset (yr)/SCCHPV typeSkin lesions (location)SCCReference
P1 A1.viii.4 50 12/no Unknown Head and hands No Rueda (1993)  
P2 A1.viii.7 47* 6/18 8, 17, 20 Head, neck, trunk, hands, legs, and feet Forehead Rueda (1993), case 11 
P3 A1.viii.10 46 7/13 8, 20 Head, trunk, legs, arms, and hands Forehead, lips Rueda (1993), case 12 
P4 A1.viii.12 44 7/no Head and trunk No Rueda (1993)  
P5 A1.viii.15 37 5/17 8, 20 Head, trunk, hands, and upper legs Forehead Rueda (1993), case 13 
P6 A1.vi.13 47* 1/15 Unknown Head, trunk, hands, and legs Nose, trunk Rueda (1993), case 6 
P7 A1.viii.30 47 5/10 5, 17, 36 Head, trunk, arms, hands, and legs Canthus, nose Rueda (1993), case 7 
P8 A2.ii.1 78* 1/47 5, 8, 20, 24 Head, trunk, and extremities Forehead Rueda (1993) and Rueda and Rodriguez (1976), case 1 both papers 
P9 A2.ii.10 Unknown 5/38 8, 9 Head, trunk, and extremities Rueda (1993) and Rueda and Rodriguez (1976), case 2 both papers 
P10 A2.ii.12 48* 1/26 Unknown Head, trunk, and extremities Forehead, lips Rueda (1993) and Rueda and Rodriguez (1976), case 3 both papers 
P11 A2.ii.13 47* 3/27 5, 8, 20 Head, trunk, and extremities Forehead Rueda (1993) and Rueda and Rodriguez (1976), case 4 both papers 
P12 B.ii.2 72* 23/43 8, 15 Head, trunk, arms, hands, and legs Forehead, check, ear Kienzler et al. (1979)  
P13 C.ii.1 71* Childhood/35 Head, neck, hands, and legs Scalp, nose Lutz (1946) and Arnold et al. (2011), case 1 both papers 
P14 C.ii.2 91* <7/52 Head, neck, and hands Nose, hand, neck, forehead, scalp Lutz (1946) and Arnold et al. (2011), case 2 both papers 
P15 D.iv.2 59 Childhood/54 Head, neck, arms, hand, knee, and lower leg Forehead, nose Imahorn et al. (2017)  
P16 E.iv.4 21* 7/unknown Unknown Face and trunk Multiple (sun-exposed skin) Saka et al. (2009)  
P17 E.iv.6 Unknown* 5/unknown Unknown Face and trunk Unknown Saka et al. (2009)  
P18 E.iv.8 Unknown* Unknown/unknown Unknown Unknown Unknown Saka et al. (2009)  
P19 E.iv.10 14 2/unknown Unknown Face and trunk Unknown Saka et al. (2009)  
P20 F.v.2 47 3/46 Unknown Head, neck, trunk, and upper and lower limb Forehead (basosquamous carcinoma) This study 
P21 F.v.3 44 2/no Unknown Head, neck, trunk, and upper and lower limb This study 
P22 F.v.7 30 5/no Unknown Head, neck, trunk, and upper and lower limb This study 
P23 F.vi.1 22 1/no Unknown Head, neck, trunk, and upper and lower limb This study 
P24 F.vi.2 21 4/no Unknown Head, neck, trunk, and upper and lower limb This study 
PatientCaseSexAge (yr)Age onset (yr)/SCCHPV typeSkin lesions (location)SCCReference
P1 A1.viii.4 50 12/no Unknown Head and hands No Rueda (1993)  
P2 A1.viii.7 47* 6/18 8, 17, 20 Head, neck, trunk, hands, legs, and feet Forehead Rueda (1993), case 11 
P3 A1.viii.10 46 7/13 8, 20 Head, trunk, legs, arms, and hands Forehead, lips Rueda (1993), case 12 
P4 A1.viii.12 44 7/no Head and trunk No Rueda (1993)  
P5 A1.viii.15 37 5/17 8, 20 Head, trunk, hands, and upper legs Forehead Rueda (1993), case 13 
P6 A1.vi.13 47* 1/15 Unknown Head, trunk, hands, and legs Nose, trunk Rueda (1993), case 6 
P7 A1.viii.30 47 5/10 5, 17, 36 Head, trunk, arms, hands, and legs Canthus, nose Rueda (1993), case 7 
P8 A2.ii.1 78* 1/47 5, 8, 20, 24 Head, trunk, and extremities Forehead Rueda (1993) and Rueda and Rodriguez (1976), case 1 both papers 
P9 A2.ii.10 Unknown 5/38 8, 9 Head, trunk, and extremities Rueda (1993) and Rueda and Rodriguez (1976), case 2 both papers 
P10 A2.ii.12 48* 1/26 Unknown Head, trunk, and extremities Forehead, lips Rueda (1993) and Rueda and Rodriguez (1976), case 3 both papers 
P11 A2.ii.13 47* 3/27 5, 8, 20 Head, trunk, and extremities Forehead Rueda (1993) and Rueda and Rodriguez (1976), case 4 both papers 
P12 B.ii.2 72* 23/43 8, 15 Head, trunk, arms, hands, and legs Forehead, check, ear Kienzler et al. (1979)  
P13 C.ii.1 71* Childhood/35 Head, neck, hands, and legs Scalp, nose Lutz (1946) and Arnold et al. (2011), case 1 both papers 
P14 C.ii.2 91* <7/52 Head, neck, and hands Nose, hand, neck, forehead, scalp Lutz (1946) and Arnold et al. (2011), case 2 both papers 
P15 D.iv.2 59 Childhood/54 Head, neck, arms, hand, knee, and lower leg Forehead, nose Imahorn et al. (2017)  
P16 E.iv.4 21* 7/unknown Unknown Face and trunk Multiple (sun-exposed skin) Saka et al. (2009)  
P17 E.iv.6 Unknown* 5/unknown Unknown Face and trunk Unknown Saka et al. (2009)  
P18 E.iv.8 Unknown* Unknown/unknown Unknown Unknown Unknown Saka et al. (2009)  
P19 E.iv.10 14 2/unknown Unknown Face and trunk Unknown Saka et al. (2009)  
P20 F.v.2 47 3/46 Unknown Head, neck, trunk, and upper and lower limb Forehead (basosquamous carcinoma) This study 
P21 F.v.3 44 2/no Unknown Head, neck, trunk, and upper and lower limb This study 
P22 F.v.7 30 5/no Unknown Head, neck, trunk, and upper and lower limb This study 
P23 F.vi.1 22 1/no Unknown Head, neck, trunk, and upper and lower limb This study 
P24 F.vi.2 21 4/no Unknown Head, neck, trunk, and upper and lower limb This study 

Information collected from listed references or oral communication with the treating physicians. Please see Fig. 1 A for individual deidentifiers. F, female; M, male; ages of death are marked with asterisks.

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