A HEREDITARY DISORDER OF CORNIFICATION AND MULTIPLE CONGENITAL-DEFECTS IN 5 ROTTWEILER DOGS

The clinical, biochemical, histological, or ultrastructural abnormalit ies of five related female Rottweiler dogs with a hereditary disorder of cornification are reported. Three of the five dogs also had multipl e noncutaneous congenital defects. Cutaneous abnormalities included ge neralized, hyperkeratotic. variably pigmented plaques, which in one do g were distributed along Blaschko's lines. Moderate to severe parakera tosis involving the follicular infundibula and ostia and focal orthoke ratosis with variable vacuolation of spinous cells was observed on his topathologic examination of all skin specimens. Supplementation with o ral zinc in two dogs and vitamin A alcohol and calcitriol in one dog d id not result in clinical or histological improvement of the hyperkera totic lesions. This disorder of cornification in dogs is similar to hu man disorders of cornification that follow the lines of Blaschko. Blas chko's lines follow a V-shape over the spine, an S-shape on the abdome n, and an axial distribution on the limbs. No related male dogs were a ffected, suggesting an X-linked dominant mode of inheritance. Many fea tures of this hereditary DOC correspond to the human condition CHILD s yndrome.