Cornified envelopes in congenital disorders of keratinization

A morphological and biochemical analysis was made of cornified envelopes isolated from patients with different congenital disorders. Nomarski contrast microscopy of the envelopes showed that their morphology was not greatly altered in several types of keratoderma and parapsoriasis, but it was grossly modified in ichthyotic disorders. The various types of ichthyoses, keratoderma palmoplantare, KID syndrome and parapsoriasis showed, after cyanogen-bromide cleavage, peptide patterns similar to those obtained from healthy subjects. In contrast, envelopes from patients with Darier's disease, congenital pachyonychia and erythrokeratoderma variabilis showed markedly different peptide patterns.