Autosomal dominant lamellar ichthyosis (ADLI) is a recently recognized keratinization disorder, which cannot be clinically and histologically distinguished from autosomal recessive lamellar ichthyosis (ARLI).  Onset is delayed until at least 3 months of age.  ADLI is characterized by large, dark brown scales covering the entire body, including flexural folds, palms and soles.  Distinctive alteration in the relative composition of the scale lipid pattern characterized by excessive amounts of free fatty acids, triglycerides, elevated n-alkanes, reduced free sterols and decreased total ceramides was found in patients with ADLI.  

The diagnosis is based on the clinical appearance of the skin. There is no cure for ichthyosis, and management is based on daily applications of emollients or keratolytics.

Lamellar ichthyosis follows an autosomal dominant inheritance.  However, the molecular basis for the condition is unknown.