Peeling skin syndrome
Encyclopedia
Peeling skin syndrome is an autosomal recessive disorder characterized by lifelong peeling of the stratum corneum
, and may be associated with pruritus, short stature, and easily removed anagen hair.
The acral form can be associated with TGM5
.
Stratum corneum
The stratum corneum is the outermost layer of the epidermis, consisting of dead cells that lack nuclei and organelles. The purpose of the stratum corneum is to form a barrier to protect underlying tissue from infection, dehydration, chemicals and mechanical stress...
, and may be associated with pruritus, short stature, and easily removed anagen hair.
The acral form can be associated with TGM5
TGM5
TGM5 is a transglutaminase enzyme.TGM5 encodes one member of a the multigene transglutaminase family. Transglutaminases are involved in protein cross-linking by catalyzing the formation of gamma-glutamyl-lysine isodipeptide bonds between adjacent polypeptides...
.