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Annals of Dermatology 2009 May; 21(2): 206~208
Annals of Dermatology 2009 May; 21(2): 206~208

A Case of Hereditary Hemorrhagic Telangiectasia
Ha Eun Lee, M.D., Chan Sagong, M.D., Kwang Yeoll Yeo, M.D., Joo Yeon Ko, M.D., Joung Soo Kim, M.D., Hee Joon Yu, M.D.
Department of Dermatology, Hanyang University College of Medicine, Seoul, Korea
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Hereditary hemorrhagic telangiectasia, also known as Osler-Weber-Rendu disease, is an autosomal dominant disorder of the fibrovascular tissue. It is characterized by the classic triad of mucocutaneous telangiectasias, recurrent hemorrhages, and familial occurrence. The cutaneous manifestation appear clinically as punctuate, linear, or splinter-like telangiectasias of the upper body, oral, and nasal mucous membranes, and nail beds. A 73-year-old woman presented with purpuric, punctuate, and tiny macules on the finger tips of both hands and the tongue. The skin lesions were discovered about 50 years previously. She had a family history of cutaneous telangiectasia. Also, she had episodes of recurrent epistaxis, gastrointestinal bleeding, and anemia. The gastroendoscopy revealed gastric angiodysplasia of the fundus and body of the stomach. The histopathologic study showed dilated capillaries lined by flat endothelial cells in the papillary dermis. From these findings, we diagnosed this case as hereditary hemorrhagic telangiectasia, which has rarely been reported in the dermatologic literature. (Ann Dermatol 21(2) 206∼208, 2009)
Annals of Dermatology 2009 May; 21(2): 206~208
Keyword : Hereditary hemorrhagic telangiectasia, Osler-Weber-Rendu disease
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