Fig. 1.

Cdsn deficiency results in stratum corneum detachment due to defective desmosome formation. (A) Macroscopic appearance of formalin-fixed Cdsn-deficient newborn mouse with skin erosions on the limbs. (B) Loss of upper epidermis due to detachment of the stratum corneum from the underlying granular layer and/or within the upper granular layers in Cdsn-deficient mouse epidermis. Dilation of capillaries beneath the affected epidermis was also noted (arrows). (Scale bar: 100 μm.) (C) Electron density of each corneodesmosome from Cdsn-deficient mice (Lower Left, arrowhead) was markedly lower than that in wild-type mice (Upper Left, arrowheads); areas included in Fig. S2B are outlined. In contrast, formation of cornified cell envelopes (arrows) was indistinguishable between wild-type and Cdsn-deficient mice. (Right) Higher magnification of the outlined areas shown in Left. (Scale bars: 100 nm).