Important

It is possible that the main title of the report Epidermal Nevus Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

• Ichthyosis Hystrix Gravior
• Inflammatory Linear Nevus Sebaceous Syndrome
• Lambert Type Ichthyosis
• Linear Nevus Sebacous Syndrome
• Linear Sebaceous Nevus Sequence
• Linear Sebaceous Nevus Syndrome
• Nevus Sebaceous of Jadassohn
• Porcupine Man
• Sebaceous Nevus Syndrome

 Disorder Subdivision

• None

 General Discussion

Epidermal Nevus Syndrome is a rare disorder characterized by distinctive birth marks (nevus) on the skin. Neurological and skeletal abnormalities may also occur. This disorder is usually apparent at birth (due to the skin lesions which are most often seen in the midface from the forehead down into the nasal area) and is often associated with seizures, mental deficiency, eye problems, bone malformations and atrophy of the brain. The exact cause of Epidermal Nevus Syndrome is not known although an autosomal dominant trait of inheritance seems to occur in approximately two thirds of the cases.