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December 1955

Angiokeratoma Corporis Diffusum

Author Affiliations

Rochester, Minn.

AMA Arch Derm. 1955;72(6):556-561. doi:10.1001/archderm.1955.03730360062007

The entity first described by Fabry in 1898 under the name "purpura haemorrhagica nodularis"1 was later given the name "angiokeratoma corporis diffusum" by him.2 The onset of the distinctive cutaneous lesions has always been noted prior to puberty. Ruiter3 and Ruiter, Pompen, and Wijers* have drawn attention to the systemic as well as the familial occurrence of the disease. Thorough study of cases by them emphasized not only the cutaneous anomaly but also visceral changes. Evidence for systemic involvement has been summarized under the title "cardiovascular-renal symptom complex." Signs and symptoms of involvement of the internal organs have been summarized by Ruiter3 as follows:

(1) vasomotor disturbances, pains and spasms in the hands, feet, and upper legs, increasing at higher temperatures and abating under the influence of cooling; (2) edema of many years standing, chiefly located in the feet and the lower parts