No abstract available.
Humans ; Hyperlipoproteinemia Type III ; Male

Generalized plane xanthoma is a group of plane xanthomas that appear as yellow to yellow-brown flat patches or slightly elevated plaques with a wide-spread distribution. The disease is frequently associated with multiple myeloma or other reticulo-endothelial malignancies and monoclonal gammopathy with unknown significance, and when accompanied by these hematologic malignancies, normolipoproteinemia is invariably associated. We present a case of generalized plane xanthoma associated with multiple myeloma and type III hyperlipoproteinemia in a 48 year-old woman, and this is the first report in Korean literature.
Female ; Hematologic Neoplasms ; Humans ; Hyperlipoproteinemia Type III ; Hyperlipoproteinemias* ; Middle Aged ; Multiple Myeloma* ; Paraproteinemias ; Xanthomatosis*

Adult ; Female ; Humans ; Hyperlipoproteinemia Type III ; pathology ; Male ; Middle Aged ; Nephrosis, Lipoid ; pathology

We report a case of type III hyperlipoproteinemia which is called a broad-beta disease. A 53 year old female patient visited our clinic for the evaluation of multiple yellowish papules on extremities and eyelids. The patient showed various types of xanthoma includiiig eruptive, tuberous, tendinous xanthomas and xanthelasma palpebrarum, xanthoma striatum palmare. The blood chemistry revealed a marked elevstion of cholesterol and triglycerides and agarose gel electrophoresis showed a single peak at prebeta and beta portion without separation. On histopathologic studies, typical foam cells were showen.
Chemistry ; Cholesterol ; Electrophoresis, Agar Gel ; Extremities ; Eyelids ; Female ; Foam Cells ; Humans ; Hyperlipoproteinemia Type III* ; Hyperlipoproteinemias ; Middle Aged ; Triglycerides ; Xanthomatosis

Xanthomas are localized lipid deposits within organs that may manifest as papules, plaques, or nodules in skin. They are commonly associated with all types of hyperlipidemia. Xanthoma striatum palmare characterized by xanthomas of the palmar creases is a rare but important diagnostic physical sign of dysbetalipoproteinemia, also known as type III hyperlipoproteinemia. Type III hyperlipoproteinemia is characterized by the early onset of cardiovascular disease and peripheral vascular disease. We describe herein the case of a 51-year-old female patient affected by xanthoma striatum palmare associated with elevated plasma levels of triglycerides and cholesterol and a lipoprotein electrophoresis pattern consistent with type III hyperlipoproteinemia.
Cardiovascular Diseases ; Cholesterol ; Electrophoresis ; Female ; Humans ; Hyperlipidemias ; Hyperlipoproteinemia Type III* ; Lipoproteins ; Middle Aged ; Peripheral Vascular Diseases ; Plasma ; Skin ; Triglycerides ; Xanthomatosis*

We report a case of type III hyperlipoproteinemia associated with xanthoma eruptivum. Xanthoma tuberosum, and xanthoma striatum palmare whose clinical symptoms have been improved by the treatment of gemfibrozil. A 31-year-old male patient visited our clinic for evaluation of multiple yellowish nodules on the elbows, multiple yellowish plaques on the buttocks and linear yellowish plaques along with the palmar creases on both palms. The blood chemistry showed an elevation of cholesterol and triglyceride. Lipoprotein electrophoresis showed broad betazone and plasma standing test showed turbid plasma. Lipoprotein ultracentrifugation showed that the ratio of VLDL cholesterol and plasma triglyceride was 0.38. According to the above findings, we diagnosed this patient as type III hyperlipoproteinemia. After 4 months of treatment with diet and gemfibrozil, the serum level of cholesterol and triglyceride were reduced to normal range. The skin lesions of both palms showed marked improvement but the skin lesions on elbows and buttocks were not much improved.
Adult ; Buttocks ; Chemistry ; Cholesterol ; Cholesterol, VLDL ; Diet ; Elbow ; Electrophoresis ; Gemfibrozil ; Humans ; Hyperlipoproteinemia Type III* ; Lipoproteins ; Male ; Plasma ; Reference Values ; Skin ; Triglycerides ; Ultracentrifugation ; Xanthomatosis*

A 55-year-old female presented with extensive yellowish eruptive plaques over both elbows and the buttocks that she had first noticed 2 years earlier. Yellowish orange discoloration of her palmar creases was noted. Her serum cholesterol and triglyceride were markedly elevated. Lipoprotein electrophoresis showed a broad beta band. On apolipoprotein E genotyping, the arginine at position 158 had been replaced by cysteine in both alleles (E2/E2). Under a diagnosis of type III hyperlipoproteinemia, combined atorvastatin and fenofibrate therapy for 2 months normalized the serum cholesterol and triglyceride levels.
Alleles ; Apolipoproteins ; Arginine ; Buttocks ; Cholesterol ; Citrus sinensis ; Cysteine ; Elbow ; Electrophoresis ; Female ; Fenofibrate ; Heptanoic Acids ; Humans ; Hyperlipoproteinemia Type III ; Hyperlipoproteinemias ; Lipoproteins ; Middle Aged ; Pyrroles ; Xanthomatosis ; Atorvastatin Calcium

A stout and obese fourty five year-old Korean man was affected with hyperlipoproteinemia type III. His skin lesion showed yellowish papular eruptive xanthomas with erythematous halo located chiefly on the trunk and extensor aspects of the extremities including both palms. In addition to the skin lesion he showed pea-sized cervical lymphnode swellings, slightly tessellated fundi and, on ECG, premature ventricular contraction. Otherwise no specific findings were seen. The laboratory examinations showed increases of both serum cholesterol and triglycerides, increased fasting blood sugar level and abnormal glucose tolerance curve with turbid color of fasting blood serum which had been kept standing at 4C for 24 hours. On the paper electrophoresis "broad beta band" blending with the pre-beta area was noted. The authors discussed laboratory characteristics, clinical manifestations and differential diagnosis of primary hyperlipoproteinemia on each type with review of the literatures of the disease.
Blood Glucose ; Cholesterol ; Diagnosis, Differential ; Electrocardiography ; Electrophoresis, Paper ; Extremities ; Fasting ; Glucose ; Hyperlipoproteinemia Type III* ; Hyperlipoproteinemias ; Lipoproteins, LDL* ; Serum ; Skin ; Triglycerides ; Ventricular Premature Complexes ; Xanthomatosis

A 61-year-old female patient presented with the type III hyperlipoproteinemia (HLP) in association with generalized eruptive and tuberous xanthomas. She had hypercholesterolemia and hypertriglyceridemia, and extensive coronary atherosclerosis. Further studies revealed a positive standing plasma test, abnormal beta-very low density lipoprotein (VLDL) on lipoprotein electrophoresis, markedly elevated very low density lipoprotein-cholesterol (VLDL-C) to plasma triglycerides (TG) ratio (0.86) and homozygosity for apolipoprotein E2. After about one year of therapy with lipid-lowering agents and diet restriction, a significant reduction of serum cholesterol and TG was observed and the yellowish orange discolorations of palmar creases disappeared from her palms.
Apolipoprotein E2 ; Cholesterol ; Citrus sinensis ; Coronary Artery Disease ; Diet ; Electrophoresis ; Female ; Humans ; Hypercholesterolemia ; Hyperlipoproteinemia Type III* ; Hypertriglyceridemia ; Lipoproteins ; Middle Aged ; Plasma ; Triglycerides ; Xanthomatosis*

Animals ; Apolipoproteins E ; deficiency ; Female ; Hyperlipoproteinemia Type III ; blood ; metabolism ; Lipid Metabolism, Inborn Errors ; blood ; metabolism ; Male ; Mice ; Mice, Inbred C57BL ; Receptors, Calcitriol ; metabolism ; Vitamin D ; blood