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angiokeratoma corporis diffusum |
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angiokeratoma corporis diffusum, an uncommon familial disease in which glycolipids are stored in many parts of the body, especially in the venous and cardiovascular systems, causing vasomotor, urinary, and cutaneous disorders and in some cases, muscular abnormalities. Characteristic signs are dilation of blood vessels in the "bathing suit areas"; edema; hypertension; cardiomegaly, especially enlargement of the left ventricle; diffuse nodularity of the skin; albumin, erythrocytes, leukocytes, and casts in the urine; and vacuoles in muscle bundles. Also called diffuse angiokeratoma, Fabry's disease, Fabry's syndrome. angiokeratoma [an″je-o-ker″ah-to´mah] a dermatosis marked by telangiectasia with secondary epithelial changes, including acanthosis and hyperkeratosis. ![]() Facial rash of angiokeratoma in a male with tuberous sclerosis. From Mueller and Young, 2001. angiokeratoma cor´poris diffu´sum an inborn error of metabolism of glycolipids characterized by purpuric skin lesions (angiokeratomas); see also fabry's disease.
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