PKP1 and ectodermal dysplasia syndrome: Among the subtypes of EB simplex, the suprabasal variants now include lethal acantholytic EB due to mutations of the gene encoding desmoplakin, the ectodermal dysplasia-skin fragility syndrome (ED-SFS) with plakophilin-1 (PKP1) deficiency and EBS superficialis of unknown genetic cause [1].