Hereditary hemorrhagic telangiectasia

This diagnosis code represents a genetic disorder characterized by malformations of blood vessels, leading to recurrent bleeding. These vascular lesions, known as telangiectasias, can occur in various organs, including the skin, mucous membranes, lungs, liver, and brain.

Assign this code when documentation confirms a diagnosis of hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu disease. This includes cases where the patient presents with epistaxis, gastrointestinal bleeding, or symptoms related to arteriovenous malformations (AVMs) in internal organs, and the genetic basis is established or strongly suspected.

• Ensure documentation specifies "hereditary" to differentiate from acquired telangiectasias.
• Code all manifestations and complications of HHT separately, such as epistaxis (R04.0) or gastrointestinal hemorrhage (K92.2).
• Do not confuse with other types of telangiectasia that are not hereditary (e.g., essential telangiectasia, I78.8).