Lethal Hereditary Vascular Disorders: Osler-Weber-Rendu, Ataxia-Telangiectasia, and Fabry’s Disease


Mucocutaneous vascular ectasia, otherwise referred to as telangiectasia, can be an important harbinger of serious systemic disease. Among a variety of acquired conditions, including hepatic cirrhosis that are responsible for their development, are a heterogeneous group of inherited conditions that entail the development of multiple cutaneous and mucous membrane vascular lesions associated with other life-threatening complications. This chapter deals with the clinical and pathologic features of three such conditions, namely Osler-Weber-Rendu syndrome, ataxiatelangiectasia, and Fabry’s disease.


Arteriovenous Malformation Fabry Disease Cerebellar Ataxia Actinic Keratosis Hereditary Hemorrhagic Telangiectasia 
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