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November 1960

Angiokeratoma Corporis Diffusum

Author Affiliations


Dermatology Service (Major Vineyard) and Department of Medicine (Captain Kamin), Walter Reed General Hospital, Washington, D.C.

Arch Dermatol. 1960;82(5):817-819. doi:10.1001/archderm.1960.01580050159024

In 1898, Fabry1 reported a previously undescribed cutaneous disorder which has since been termed angiokeratoma corporis diffusum. Over the next few decades, dermatological periodicals contained reports of other examples of this condition. It slowly became apparent that this disorder was not limited to the integument, and that the cutaneous lesions merely represent the earliest and most easily demonstrable evidence of a sometimes fatal syndrome involving multiple organs.2,3 We wish to report an additional case of this syndrome and briefly review the characteristic manifestations.

The cutaneous lesions are the earliest aberration and have been present in all reported cases. They consist of minute macules and papules which may or may not have an overlying scale. The lesions are dark blue-red to purple in color and may appear black in older individuals. Slight, if any, change on diascopic pressure is characteristic. They are scattered over the entire body but tend

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