An X-linked recessive variety of ichthyosis vulgaris different from the X-linked ichthyosis of Wells and Kerr

Abstract

Pedigree analysis of ichthyosiz vulgaris in an extensive North German kindrcd revealed X-linked recessive inheritance. Only males were affected; heterozygous females had no sign of this dermatosis. Clinically and histologically, the disease resembled the autosomal dominant form. The differential diagnosis between this X-linked recessive ichthyosis vulgaris, ichthyosiz congenita mitis, and X-linked ichthyosis of the Wells-Kerr type is discussed. We conclude from our qtudy that ichthyosis vulgariz is genetically heterogeneous.

Dominant ichthyosis vulgaris with an ultrastructuraally normal granular layer

“…In conclusion, the present and previous genetic and morphologic analyses of ichthyosis vulgaris suggest that the appearance of the granular layer alone cannot always distinguish between the dominant and sexlinked types. The dominant may have a normal granular layer, as in this report, and the sex-linked type may have a decreased granular layer, as shown by Vibrans & Altwein (1970). In view of these observations, we believe that the appearance of the stratum granulosum must be used in conjunction with the pedigree analysis and clinical features in order to distinguish the different types of ichthyosis.…”

“…Passarge et aI. (1971), and another large family studied by Vibrans & Altwein (1970). It is of interest to note that Vibrans & Altwein have reported a decreased granular layer in the skin biopsy.…”

Ichthyosis vulgaris showing features of the autosomal dominant and the X‐linked recessive variants in the same family*

“…Passarge et al (1971) described a kindred with ichthyosis which was genetically of the X-linked type, but which showed clinical and histological findings intermediate between AD1 and XLI. The ichthyosis in the kindred observed by Vibrans & Altwein (1970) also revealed a recessive X-linked mode of inheritance. However, clinically and histologically it resembled ADI.…”