Which skin exam findings are characteristic of Osler-Weber-Rendu disease (OWRD) (hereditary hemorrhagic telangiectasia [HHT])?

Updated: Oct 06, 2020
  • Author: Klaus-Dieter Lessnau, MD, FCCP; Chief Editor: Vincent Lopez Rowe, MD  more...
  • Print
Answer

Skin lesions begin as dark red lines or as punctate, pulsating vascular papules the size of match heads. These may be found on the skin, oral mucosa, nasal mucosa, and conjunctiva. [39] More rarely, skin lesions are star-shaped and 1-3 mm in diameter; alternatively, they are nonpulsating telangiectases resembling spider angiomas. (See the images below.)

Typical manifestations in patient with Osler-Weber Typical manifestations in patient with Osler-Weber-Rendu disease (ie, hereditary hemorrhagic telangiectasia) with red nodules and starry telangiectasia on cheeks.
Close-up view of typical manifestations in patient Close-up view of typical manifestations in patient with Osler-Weber-Rendu disease (ie, hereditary hemorrhagic telangiectasia) with red nodules and starry telangiectasia on lips.
Close-up view of typical manifestations in patient Close-up view of typical manifestations in patient with Osler-Weber-Rendu disease (ie, hereditary hemorrhagic telangiectasia) with red nodules and starry telangiectasia on cheeks.

The lesions blanch partially with pressure. Fine telangiectases may be difficult to appreciate in patients with anemia. Lesions often are conspicuous in the nail beds.

Half of patients manifest cutaneous lesions by age 30 years, though lesions may arise during the teenage years. [6] The face, lips and mouth, nares, tongue, ears, hands, chest, and feet are most often affected, in descending order of frequency and in any combination. Lesions are multiple and may be of cosmetic concern, and the number of lesions may increase with age. Bleeding is rarely clinically significant.

Almost invariably, all of the mucous membranes are involved, including membranes throughout the GI, respiratory, and urinary tracts and those in the nasal septum, oral cavity, and nasopharynx.


Did this answer your question?
Additional feedback? (Optional)
Thank you for your feedback!