FAMILIAL HAEMORRHAGIC TELANGIECTASIA * 20 Cases treated with Systemic Oestrogen

THE condition of familial haemorrhagio telangiectasia (Osler-Rendu-Weber disease), characterized typically by the triad of multiple telangiectases, haemor-rhage, and a history of familial occurrence, is supposedly quite rare: However, as over 98 per cent, of cases present primarily with epistaxis, it is not unlikely that the bleeding is frequently treated without the underlying pathology being appreciated. Historical Note It is probable that the first case was reported in 1864 by Gamen Sutton who-described some curious cases of recurrent epistaxis associated with internal haemorrhages and telangiectases of the skin. The following year Benjamin Guy Babington recorded in the Lancet the clinical history of a family which suffered from an hereditary tendency to epi-staxis. Violent and habitual epistaxis was observed in five consecutive genera-tions, causing death in two cases and affecting both male and female members of the family. Although no reference was made to the presence or absence of telangiectases it is most probable that the condition desoribed was that of familial haemorrhagic telangiectasia. Rendu in 1896 reported a case of a 52-year-old man who suffered from repeated epistaxis and who had small superficial angiomata on the nTrin of the face, neck,, thorax, and mucous membrane of the mouth. This patient's mother and one brother also had repeated epistaxis and Rendu termed this pseudo-haemo-philia. In 1901 Sir William Osier wrote a paper entitled 'A family form of recurring epistaxis associated with multiple telangiectases of the akin and mucous mem-brane ' • He described all the manifestations of the condition with the exception of pulmonary arteriovenous aneurysm, which was not recognized in any case prior to 1940. Weber in 1907 and Hanes in 1909 made further contributions towards clari-fying the clinical and pathological characteristics of the disease. It was Hanes-who suggested the descriptive terminology of familial haemorrhagic telangiec-tasia instead of the eponym 'Osler-Rendu-Weber ' disease