Xanthomata

Ahrens, E.H. jr., Knnkel, H.G. The relationship between serum lipids and skin xanthomata in eighteen patients with primary biliary cirrhosis. J. Clin. Invest. 1949 28 1565-1574... 

Muller C. Xanthomata, hypercholesterolemia, angina pectoris. Acta Med Scand 1938 75. 

Initially recorded with rare paraproteins (B23), mainly of the IgA class, this has also been associated with IgG and IgM. Usually the total serum cholesterol is raised, but in one case it has been within normal limits curiously it is subnormal in most patients with IgA-myelomatosis without xanthoma (S7). The xanthomata are typically of the soft eruptive variety and contain complexes of the paraprotein and /8-Iipo-protein. Beaumont (B8) has collected evidence suggesting that the paraproteins are antibodies to the 8-apoprotein. Occasionally excess complexes can result in viscosity syndrome (Section 7.5.6). If a lipid stain is used, the paraprotein band is positive. In such cases, regrettably. Potter has been unable to relate the antibody activity to phosphoryl-choline (P13). Cytotoxic treatment can reduce the serum levels of lipid and paraprotein. 

Figures in parentheses indicate the number of subjects in each group. In essential hypercholesterolemia (type II), familial history is unclear, tendon xanthomata are absent, and serum cholesterol is usually only moderately increased. 

Another group of hypercholesterolemic (type II) patients, indicated in Table II by the term essential hypercholesterolemia, was also studied. These patients differed from familial hypercholesterolemia patients in that the family history was less clear, serum cholesterol was less elevated, and xanthomata were not present. Hypercholesterolemia may be primarily caused by environmental, primarily dietary, factors. Bile acid production in this group is less significantly reduced than in the familial group, and the relative catabolism of cholesterol by way of bile acids is within normal limits. Sodhi (151) observed in this type of hypercholesterolemia a markedly low fecal bile acid excretion. 

There are two major indications for the lowering of senxm lipids. One is the reduction of severe hyperlipidemia to (a) prevent lipid deposits (xanthomata) that can be disfiguring and occasionally painful and (b) elimination of abdominal pain and pancreatitis due to high lipid levels. The other rationale to reduce serum lipids is the strongly... 

Asboe-Hansen, G. and Kaalund-Jorgensen, O., Systemic mast-cell disease involving skin, liver, bone-marrow, and blood associated with disseminated xanthomata, Acta haematol. 16, 273 (1956). 

Almost as characteristic for EFH are xanthomata plana. They consist of flat, yellow to red-brown plaques which have a velvety texture, may be confluent and... 

Xanthomata plana (xanthelasmas) are not considered characteristic of hyperlipemia their occurrence however has been described by Malmros and coworkers (1954), Adlersberg (1955), Schettler (1955), Matras (1956), Schirren (1957) and Haensch (1958). The presence of xanthelasmas in EHL seem either to indicate that one is dealing with a combination with essential hypercholesterolemia or that they are unrelated to the disease since they do occur in a significant percentage of normolipemic subjects. 

Pancreas A case report of 5-year-old girl involved VPA-induced severe acute pancreatitis with necrosis. Nine years later, at the age of 14, she developed eruptive xanthomata, hypertriglyceridemia, and was diagnosed with diabetes. This was believed to occur as a result of inadequate pancreatic tissue to support increasing insulin requirements during growth and adolescence [186 ]. 

Jetha MM, Fiorillo L. Xanthomata and diabetes in an adolescent with familial dysbetalipoproteinemia 9 years after valproate-induced pancreatitis. Pediatr Diabetes August 2012 13(5) 444-7. 

X = tendon xanthomata, H = homozygous, M = mother, F = father second values after cholestyramine treatment. include fecal bile acids of chenodeoxycholic acid origin values collected from ref. 25-34 bile acids measured in, 22 subjects Two present cases and patients from ref. 26,28,29.°Mainly from... 

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