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

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Deadly Dermatologic Diseases

Abstract

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 (OWR) syndrome, ataxia-telangiectasia (AT), and Fabry’s disease (FD).

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OWR

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Correspondence to Michael B. Morgan .

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Michael, G., Morgan, M.B. (2016). Lethal Hereditary Vascular Disorders: Osler-Weber-Rendu Syndrome, Ataxia-Telangiectasia, and Fabry’s Disease. In: Crowe, D., Morgan, M., Somach, S., Trapp, K. (eds) Deadly Dermatologic Diseases. Springer, Cham. https://doi.org/10.1007/978-3-319-31566-9_15

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  • DOI: https://doi.org/10.1007/978-3-319-31566-9_15

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