Figure 3.

Histology of skin and oral mucosa of neonatal mice injected with PF and PV IgG. (a) Skin from a wild-type mouse injected with (10 mg) PF982 (anti-Dsg1) IgG shows superficial blisters. (b) Mucous membrane from a wild-type mouse injected with (10 mg) PF982 IgG shows no blisters. Skin from a DSG3^null mouse injected with (10 mg) PF982 IgG reveals deep suprabasilar blisters (c) and extensive acantholysis (d). (e) Mucous membrane from a DSG3^null neonatal mouse injected with (10 mg) PF982 IgG shows deep suprabasilar blisters. (c–e) These results demonstrate that in DSG3^null mice, in which there is no Dsg3 to compensate, PF IgG causes deep suprabasilar blisters in both the skin and mucous membranes. (f) Skin from a DSG3^null mouse injected with (10 mg) PV3014 (anti-Dsg1 and anti-Dsg3) IgG shows suprabasilar blisters. (g) Skin from a wild-type mouse injected with (26 mg) PV3024 (anti-Dsg3) IgG shows no blisters. (h) Skin from a wild-type mouse injected with (10 mg) PV3014 (anti-Dsg1 and anti-Dsg3) IgG show suprabasilar blisters. (i) Mucous membrane from a wild-type mouse injected with (10 mg) PV3014 IgG shows suprabasilar blisters. (j) Skin from a wild-type mouse injected with (10 mg) PV3024 IgG and (1 mg) PF982 IgG shows suprabasilar blisters. (f–j) These results demonstrate that anti-Dsg1 antibodies in PV are pathogenic and that both anti-Dsg1 and anti-Dsg3 antibodies are necessary for efficient blister formation in PV.