Congenital bullous ichthyosiform erythroderma

This code represents a severe, inherited skin disorder characterized by widespread redness (erythroderma) and blistering (bullae) present at birth or shortly thereafter. The skin is fragile and prone to recurrent blistering, leading to thickened, scaly patches (ichthyosis) over time. It is a form of epidermolytic ichthyosis.

Use this code for patients diagnosed with congenital bullous ichthyosiform erythroderma, also known as epidermolytic hyperkeratosis. Documentation should clearly state the diagnosis of this specific congenital ichthyosis, often noting the presence of blistering and erythroderma from birth.

• Differentiate from other forms of ichthyosis; this code is specific to the bullous, erythroderma type.
• Review genetic testing results if available, as they can confirm the diagnosis of epidermolytic ichthyosis.
• Consider additional codes for associated complications such as infections, dehydration, or growth failure.