Ichthyosis congenita

This code represents a group of rare genetic skin disorders characterized by generalized, persistent scaling and thickening of the epidermis present at birth or shortly thereafter. These conditions result from defects in skin barrier function, leading to dry, scaly skin that can range in severity from mild flaking to severe, plate-like scales.

Use this code for diagnoses of ichthyosis that are explicitly documented as congenital, indicating the condition's presence from birth. This includes various forms like lamellar ichthyosis, non-bullous congenital ichthyosiform erythroderma, and harlequin ichthyosis, when the congenital onset is specified. Documentation should clearly state "congenital ichthyosis" or a specific type known to be congenital.

• Differentiate from acquired forms of ichthyosis, which are coded elsewhere.
• Ensure documentation specifies "congenital" to support this code; otherwise, a more general or unspecified ichthyosis code may be appropriate.
• Consider additional codes for associated complications or manifestations, such as ectropion or growth retardation, if documented.