Osler病の1例

OSLER'S DISEASE

I．はじめに

　Osler病（遺伝性出血性血管拡張症）は1901年にOsler^1）が報告して以来，1つの確立された疾患とされている。その主要症状は多発性毛細血管拡張症と出血傾向で家族的に発生するとされている。

　本邦においては昭和17年に大久保^2）の報告以来，およそ22家系に見られるにすぎない。最近，Osler病の3主徴（①多発性毛細血管拡張，②出血傾向，③家族的発生）を有する15歳，女子の例を経験したのでここに報告する。

A 15-year-old girl noticed bleeding from the lip and an angiomatous eruption on the face, dorsa of the hands and lip in 1963. In Dec., 1968, she had an attack of cramps of the left upper and lower extremities. At the end of 1968, she had an episode of hematuria.

Her paternal grandfather and father had a telangiectatic eruption. At her first visit in 1968, she had red, punctate or branched. telangiectatic macules in the retroauricular regions, dorsa of the hands, forearms, lips and tongue.

There was no particular finding in the fundus oculi, nasopharyngeal membrane, and mem-brane of the bladder. The liver and spleen could not be palpated.

Laboratory tests revealed no abnormalities in hematologic studies, blood coagulation factors, and liver functions, but proved microhematuria.

Histologic specimen showed branched vessels with rather abundant muscular components in the deeper layer of the dermis. The wall of some dilated vessels was thin and endothelial cells could not be recongnized definitely.