• The association between congenital large-vessel arteriovenous (AV) fistulae of the extremities with Kaposiform acroangiomatosis is well established. Based on pathogenetic considerations, it is likely that many benign, cutaneous angiomatoses represent minute or microscopic AV fistulae. Combined large vessel and small vessel congenital AV fistulae of the extremity would result in the previously mentioned syndrome.
(Arch Dermatol 112:1135-1138, 1976)