Swensson B, Swensson O, Häring G
Klinik für Ophthalmologie der Christian-Albrechts-Universität zu Kjel.
Klin Monbl Augenheilkd. 1998 Feb;212(2):116-9. doi: 10.1055/s-2008-1034844.
Widespread idiopathic telangiectasia (generalized essential telangiectasia) is a rare skin disorder characterized by the development and gradual spreading of telangiectases. The condition tends to affect women in their midthirties. For no apparent reason telangiectases start to appear to the lower extremities and progress steadily to involve the skin of the trunk, the arms, and the face. General health is not affected by the condition and standard laboratory tests consistently yield normal results.
In February 1997 a 78-year-old lady was admitted for treatment of cataracta corticonuclearis of her left eye. Complete ophthalmological and dermatological examinations were performed. She presented marked conjunctival telangiectases of both eyes and widespread cutaneous telangiectases involving her face, trunk, arms, and legs. Complete blanching of lesional skin was observed on diascopy. The Rumpel-Leede-test was normal. Cutaneous and conjunctival changes appeared not to be associated with internal disease or bleeding abnormalities.
The patient presented here shows widespread idiopathic telangiectasia with marked conjunctival involvement. Ocular changes rarely have been reported in patients with generalized essential telangiectasia to date. Concomittant conjunctival and cutaneous telangiectases may be seen in other conditions such as hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber disease) and ataxia telangiectasia (Louis-Bar syndrome). The former shows an associated bleeding abnormality and is transmitted autosomal dominantly. The latter presents associated neurological signs such as cerebellar ataxia, strabism, nystagmus, apraxia, and mental retardation.
广泛特发性毛细血管扩张症（全身性特发性毛细血管扩张症）是一种罕见的皮肤疾病，其特征为毛细血管扩张的形成及逐渐蔓延。该病倾向于影响35岁左右的女性。毫无明显原因，毛细血管扩张开始出现在下肢，并稳步发展至累及躯干、手臂及面部皮肤。一般健康状况不受该疾病影响，标准实验室检查结果始终正常。
1997年2月，一名78岁女性因左眼皮质核性白内障入院治疗。进行了全面的眼科和皮肤科检查。她双眼结膜有明显的毛细血管扩张，面部、躯干、手臂及腿部有广泛的皮肤毛细血管扩张。玻片压诊时可见皮损皮肤完全变白。束臂试验正常。皮肤和结膜改变似乎与内科疾病或出血异常无关。
此处报告的患者表现为广泛特发性毛细血管扩张症且结膜受累明显。迄今为止，全身性特发性毛细血管扩张症患者很少有眼部改变的报道。在其他疾病如遗传性出血性毛细血管扩张症（伦迪 - 奥斯勒 - 韦伯病）和共济失调毛细血管扩张症（路易斯 - 巴尔综合征）中可能会同时出现结膜和皮肤毛细血管扩张。前者伴有出血异常，呈常染色体显性遗传。后者伴有小脑共济失调、斜视、眼球震颤、失用症及智力发育迟缓等神经系统体征。
