Lamellar ichthyosis

This code represents a rare, severe form of congenital ichthyosis characterized by large, dark, plate-like scales that cover the entire body at birth. The scales are typically shed within the first few weeks of life, revealing persistent generalized erythema and fine, adherent scales. It results from genetic mutations affecting epidermal differentiation.

Use this code for patients diagnosed with lamellar ichthyosis, a specific type of autosomal recessive congenital ichthyosis. Documentation should clearly state the diagnosis of lamellar ichthyosis, often confirmed by genetic testing or characteristic clinical presentation. This code is appropriate for initial diagnosis and subsequent encounters for management of this chronic condition.

• Differentiate from other forms of ichthyosis; avoid using non-specific ichthyosis codes when lamellar ichthyosis is confirmed.
• Consider additional codes for associated complications such as ectropion (H02.1x), anhidrosis (R61.0), or growth retardation (R62.8x).
• Ensure the medical record explicitly states "lamellar ichthyosis" to support the specificity of this code.