I78.0

Applicable To

• Rendu-Osler-Weber disease

Approximate Synonyms

• Hereditary hemorrhagic telangiectasia syndrome

• Osler hemorrhagic telangiectasia syndrome

Clinical Information

• An autosomal dominant hereditary disorder caused by mutations in the acvrl1, eng, and smad4 genes. It is characterized by the presence of telangiectasias in the skin, mucous membranes, lungs, brain, liver, and gastrointestinal tract. It results in hemorrhages from the affected areas.

• An autosomal dominant vascular anomaly characterized by telangiectases of the skin and mucous membranes and by recurrent gastrointestinal bleeding. This disorder is caused by mutations of a gene on chromosome 9q3) which encodes endoglin, a membrane glycoprotein that binds transforming growth factor beta.

Details

• I78.0 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.

• The 2023 edition of ICD-10-CM I78.0 became effective on October 1, 2022.

• This is the American ICD-10-CM version of I78.0 – other international versions of ICD-10 I78.0 may differ.