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Disorders of Keratinization

»	What are the ichthyosiform dermatoses?
»	What does “ichthyosis” mean?
»	How common are the congenital ichthyoses? How are they inherited?
»	What features help differentiate the most common inherited ichthyoses?
»	What genetic defect is responsible for X-linked ichthyosis (XLI)?
»	What additional phenotypes may patients with XLI contiguous gene syndromes exhibit?
»	What important birth history may be obtained in patients with XLI?
»	Name the hereditary syndromes presenting with ichthyosis as a component.What is a collodion baby?
»	What is a harlequin fetus?
»	Name several conditions associated with acquired ichthyosis.
»	Are laboratory tests helpful in the diagnosis of ichthyoses?
»	Is prenatal diagnosis of congenital ichthyosis possible?
»	How is ichthyosis treated?
»	What is Hailey-Hailey disease?
»	How does Hailey-Hailey disease present?
»	Do any factors exacerbate the skin changes of Hailey-Hailey?
»	Which diseases may be confused with Hailey-Hailey disease?
»	How is Hailey-Hailey disease treated?
»	What is Darier’s disease?
»	How is Darier’s disease diagnosed?
»	Are there any nail or hair changes in Darier’s disease?
»	Is Darier’s disease difficult to treat?
»	What is a collodion baby?

What additional phenotypes may patients with XLI contiguous gene syndromes exhibit?

Hypogonadotropic hypogonadism and anosmia (Kallman’s syndrome), chondrodysplasia punctata, short stature, and/or ocular albinism may be present in these patients in addition to X-linked ichthyosis.