PLEC and Menkes disease: Likewise, in skin of plectin-null mice as well as of EBS-MD and EBS-Ogna patients with P1a deficiencies, HDs were found to be hypoplastic and numerically reduced (Gache et al. 1996; Andrä et al. 1997; McMillan et al. 1998; Koss-Harnes et al. 2002).