Hereditary Hemorrhagic Telangiectasia: Review of 102 Cases and Presentation of an Innovation to Septodermoplasty

Abstract

SINCE 1865 when Babington¹ first called attention to this unusual form of epistaxis in a brief letter to the editor of Lancet, physicians have written on the history, etiology, pathology, clinical syndrome, and treatment of hereditary hemorrhagic telangiectasia. Rendu² was the first to associate telangiectasia with familial epistaxis and separate it from hereditary hemophilias. Osler³ in 1901 gave the first complete clinical description of the disease, pointing out the familial occurrence and the total body distribution of hemorrhagic telangiectasia. Six years later Weber discussed the condition, and he later emphasized that this was a "congenital-developmental" disease which manifests itself in later life.⁴ In 1909 Hanes,⁵ the forgotten man in Rendu-Osler-Weber disease, was the first to give the histopathologic description of the lesion and produce color plates of persons with typical lesions. His classic description of the skin lesions of hereditary hemorrhagic telangiectasia spoke of "the