I would like to thank Dr Partsch for the interest that he has shown in our article. At the time we wrote the article it was indeed true that kaposiform congenital acroangiomatosis associated with arteriovenous (AV) fistula had been reported on only four occasions in the world literature. Since that time four additional publications, cited by Dr Partsch, and a letter to the editor by Amon,1 have been written on the same topic.Reid2 indicated that it is a marvel, not that congenital AV fistula occur, but that they do not occur more often in view of the complex embryologic processes involved in the differentiation of the definitive peripheral vascular system. A greater than 50% association of benign cutaneous angiomatosis with large vessel AV fistula has been observed.3,4 I would suspect that this association had not been generally appreciated. However, the incidence of seven publications
Rusin LJ. Congenital Acroangiomatosis With Arteriovenous Fistula-Reply. Arch Dermatol. 1977;113(10):1461. doi:10.1001/archderm.1977.01640100138042
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