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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?

How does Hailey-Hailey disease present?

Fig. 4.5 Hailey-Hailey disease showing a patch of minute vesicles, erosions, and crusting in the antecubital fossa.

Fig. 4.4 Patient with Hailey-Hailey disease demonstrating characteristic involvement of flexural areas of inflammatory crease.

Skin changes consisting of localized patches of minute vesicles and erosions start in areas of friction, usually the neck, groin, and axilla (Figs. 4-4 and 4-5). Patches spread peripherally with serpiginous borders and show central healing with hyperpigmentation or granular vegetations. Onset of lesions is often delayed until the second or third decade. Associated symptoms include itching, pain, and foul odor.