2022 ICD-10-CM Diagnosis Code I78.0

Hereditary hemorrhagic telangiectasia

    2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code
  • I78.0 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2022 edition of ICD-10-CM I78.0 became effective on October 1, 2021.
  • This is the American ICD-10-CM version of I78.0 - other international versions of ICD-10 I78.0 may differ.
Applicable To
  • Rendu-Osler-Weber disease
The following code(s) above I78.0 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to I78.0:
  • I00-I99
    2022 ICD-10-CM Range I00-I99

    Diseases of the circulatory system

    Type 2 Excludes
    • certain conditions originating in the perinatal period (P04-P96)
    • certain infectious and parasitic diseases (A00-B99)
    • complications of pregnancy, childbirth and the puerperium (O00-O9A)
    • congenital malformations, deformations, and chromosomal abnormalities (Q00-Q99)
    • endocrine, nutritional and metabolic diseases (E00-E88)
    • injury, poisoning and certain other consequences of external causes (S00-T88)
    • neoplasms (C00-D49)
    • symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)
    • systemic connective tissue disorders (M30-M36)
    • transient cerebral ischemic attacks and related syndromes (G45.-)
    Diseases of the circulatory system
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.
ICD-10-CM I78.0 is grouped within Diagnostic Related Group(s) (MS-DRG v39.0):
  • 299 Peripheral vascular disorders with mcc
  • 300 Peripheral vascular disorders with cc
  • 301 Peripheral vascular disorders without cc/mcc

Convert I78.0 to ICD-9-CM

Code History
  • 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
  • 2017 (effective 10/1/2016): No change
  • 2018 (effective 10/1/2017): No change
  • 2019 (effective 10/1/2018): No change
  • 2020 (effective 10/1/2019): No change
  • 2021 (effective 10/1/2020): No change
  • 2022 (effective 10/1/2021): No change

Diagnosis Index entries containing back-references to I78.0:
  • Angioma - see also Hemangioma, by site
    • hemorrhagicum hereditaria I78.0
  • Angiomatosis Q82.8
    ICD-10-CM Diagnosis Code Q82.8

    Other specified congenital malformations of skin

      2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code POA Exempt
    Applicable To
    • Abnormal palmar creases
    • Accessory skin tags
    • Benign familial pemphigus [Hailey-Hailey]
    • Congenital poikiloderma
    • Cutis laxa (hyperelastica)
    • Dermatoglyphic anomalies
    • Inherited keratosis palmaris et plantaris
    • Keratosis follicularis [Darier-White]
    Type 1 Excludes
    • Ehlers-Danlos syndromes (Q79.6-)
    • hemorrhagic familial I78.0
    • hereditary familial I78.0
  • Babington's disease I78.0 (familial hemorrhagic telangiectasia)
  • Disease, diseased - see also Syndrome
    • Babington's I78.0 (familial hemorrhagic telangiectasia)
    • Goldstein's I78.0 (familial hemorrhagic telangiectasia)
    • Osler-Rendu I78.0 (familial hemorrhagic telangiectasia)
    • Rendu-Osler-Weber I78.0 (familial hemorrhagic telangiectasia)
  • Epistaxis (multiple) R04.0
    ICD-10-CM Diagnosis Code R04.0


      2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code
    Applicable To
    • Hemorrhage from nose
    • Nosebleed
    • hereditary I78.0
  • Goldstein's disease I78.0 (familial hemorrhagic telangiectasia)
  • Osler I78.0 (-Weber)
  • Rendu-Osler-Weber disease or syndrome I78.0
  • Syndrome - see also Disease
    • Osler-Weber-Rendu I78.0
    • Rendu-Osler-Weber I78.0
  • Telangiectasia, telangiectasis (verrucous) I78.1
    ICD-10-CM Diagnosis Code I78.1

    Nevus, non-neoplastic

      2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code
    Applicable To
    • Araneus nevus
    • Senile nevus
    • Spider nevus
    • Stellar nevus
    Type 1 Excludes
    Type 2 Excludes
    • familial I78.0
    • hemorrhagic, hereditary I78.0 (congenital) (senile)
    • hereditary, hemorrhagic I78.0 (congenital) (senile)
  • Weber-Osler syndrome I78.0

ICD-10-CM Codes Adjacent To I78.0
I77.79 Dissection of other specified artery
I77.8 Other specified disorders of arteries and arterioles
I77.81 Aortic ectasia
I77.810 Thoracic aortic ectasia
I77.811 Abdominal aortic ectasia
I77.812 Thoracoabdominal aortic ectasia
I77.819 …… unspecified site
I77.89 Other specified disorders of arteries and arterioles
I77.9 Disorder of arteries and arterioles, unspecified
I78 Diseases of capillaries
I78.0 Hereditary hemorrhagic telangiectasia
I78.1 Nevus, non-neoplastic
I78.8 Other diseases of capillaries
I78.9 Disease of capillaries, unspecified
I79 Disorders of arteries, arterioles and capillaries in diseases classified elsewhere
I79.0 Aneurysm of aorta in diseases classified elsewhere
I79.1 Aortitis in diseases classified elsewhere
I79.8 Other disorders of arteries, arterioles and capillaries in diseases classified elsewhere
I80 Phlebitis and thrombophlebitis
I80.0 Phlebitis and thrombophlebitis of superficial vessels of lower extremities
I80.00 Phlebitis and thrombophlebitis of superficial vessels of unspecified lower extremity

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.