We recently had the opportunity to study a patient with a rare condition hitherto unrecognized in American literature and one that may be readily confused with other disorders, especially purpura and hereditary telangiectasia. This led us to review the literature concerned with a form of "angiokeratoma" first described by Fabry in 1898 under the title of "purpura hemorrhagica nodularis" and more recently claimed in European reports to represent a systemic condition.
HISTORICAL REVIEW
The term "angiokeratoma" was introduced by Mibelli* to designate small raised partially hyperkeratotic vascular aneurysms in the skin of the extensor surfaces of the distal phalanges of the hands and feet and, more rarely, of other exposed areas of the body. The lesion Mibelli described is associated with exposure to cold and chilblains. The same term has been used to designate somewhat similar lesions occurring mainly over the scrotum in elderly persons; this is the so-called angiokeratoma