A Rare Case of Very Severe Hypertriglyceridemia with Eruptive Xanthomatosis

A BSTRACT Hypertriglyceridemia is increasingly becoming common in the medical world but very severe hypertriglyceridemia above 2000 mg/dL is rare. It is usually multifactorial and can be caused by primary genetic mutations, secondary chronic diseases, or a combination of both. The physician must recognize eruptive xanthomatosis as a warning sign for severe hypertriglyceridemia. Hypertriglyceridemia increases the risk of pancreatitis and cardiovascular disease and requires lowering by lifestyle change and pharmacotherapy. Furthermore, etiology needs to be addressed. Gestational onset of hypertriglyceridemia is a rare entity that occurs due to a change in plasma lipid level during pregnancy with e background of genetic mutation and poses a great risk to mother and fetus, such as preeclampsia, hyperviscosity syndrome, pancreatitis, fetal macrosomia, and fetal loss. Elevated triglyceride (TG) level in pregnancy is an important risk factor for postpartum hypertriglyceridemia and can be long-lasting. This is a case of a 42-year-old female with diabetes mellitus, hypothyroidism, mixed dyslipidemia and a past history of severe gestational hypertriglyceridemia who presented to us with multiple pearly reddish-yellow papules with a diameter of <5 mm on the trunk and both upper limbs and a TG level of 7031 mg/dL, with a dramatic reduction in TG levels in response to insulin infusion in addition to dietary restriction, statin, and other TG-lowering therapy.