Disorders of Keratinization

Chapter 650 Disorders of Keratinization




Disorders of Cornification


Disorders of cornification (ichthyoses) are a primary group of inherited conditions characterized clinically by patterns of scaling and histopathologically by hyperkeratosis. They are usually distinguishable on the basis of inheritance patterns, clinical features, associated defects, and histopathologic changes (Table 650-1).




Harlequin Ichthyosis







Collodion Baby







Lamellar Ichthyosis and Congenital Ichthyosiform Erythroderma (Nonbullous Congenital Ichthyosiform Erythroderma)


Lamellar ichthyosis and congenital ichthyosiform erythroderma (nonbullous congenital ichthyosiform erythroderma) are the most common types of autosomal recessively inherited ichthyosis. Both forms are present soon or shortly after birth. Most infants with these forms of ichthyosis present with erythroderma and scaling; but among collodion babies, most turn out to have one of these ichthyosis variants.







Ichthyosis Vulgaris






Jun 18, 2016 | Posted by in PEDIATRICS | Comments Off on Disorders of Keratinization
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