Eruptive xanthomas in a patient with soft-drink diabetic ketosis and apolipoprotein E4/2

Endocr J. 2019 Jan 28;66(1):107-114. doi: 10.1507/endocrj.EJ18-0356. Epub 2018 Nov 2.

Abstract

Soft-drink diabetic ketosis, characterized by acute onset ketosis induced by excessive ingestion of sugar-containing drinks, is often seen in obese, young patients, even with undiagnosed type 2 diabetes. We herein report a 15-year-old obese patient with the apolipoprotein E4/2 phenotype, in whom eruptive xanthomas lead to a diagnosis of soft-drink diabetic ketosis. He developed multiple asymptomatic yellowish papules on the auricles, back, buttocks and the extensor surfaces of the elbows and knees. He initially visited a dermatology clinic and his blood triglyceride and HbA1c levels were found to be 6,490 mg/dL and 16.5%, respectively. He was referred to our hospital for treatment of hyperglycemia and hypertyriglyceridemia. On admission, he had ketonuria and increased blood levels of 3-hydroxybutylate and acetoacetate. He habitually drank 1-3 litters of sweet beverages daily to quench his thirst. Therefore, "soft-drink diabetic ketosis" was diagnosed. Severe hypertriglyceridemia was considered to have been a consequence of impaired insulin action and his apolipoprotein E4/2 phenotype. We treated the diabetic ketosis and hypertriglyceridemia with intensive insulin therapy and a fat-restricted diet. At discharge, he no longer required insulin therapy and his blood glucose levels were controlled with metformin and voglibose. Along with amelioration of the hyperglycemia, triglyceride levels decreased to 247 mg/dL without administration of anti-hyperlipidemia agents. The eruptive xanthoma lesions gradually diminished in size and number and eventually disappeared by 12 months. This case provides an instructive example of eruptive xanthomas serving as a sign of severe dysregulation, not only of lipid, but also glucose, metabolism.

Keywords: Apolipoprotein E4/2; Eruptive xanthoma; Soft-drink diabetic ketosis.

Publication types

  • Case Reports

MeSH terms

  • 3-Hydroxybutyric Acid / blood
  • Acetoacetates / blood
  • Adolescent
  • Apolipoprotein E2
  • Apolipoprotein E4
  • Carbonated Beverages / adverse effects
  • Diabetes Mellitus, Type 2 / complications
  • Diabetes Mellitus, Type 2 / diagnosis*
  • Diabetes Mellitus, Type 2 / drug therapy
  • Diabetes Mellitus, Type 2 / metabolism
  • Diabetic Ketoacidosis / diagnosis*
  • Diabetic Ketoacidosis / drug therapy
  • Diabetic Ketoacidosis / etiology
  • Diabetic Ketoacidosis / metabolism
  • Diet, Fat-Restricted
  • Glycated Hemoglobin / metabolism
  • Humans
  • Hypertriglyceridemia / complications
  • Hypertriglyceridemia / diagnosis*
  • Hypertriglyceridemia / diet therapy
  • Hypertriglyceridemia / metabolism
  • Hypoglycemic Agents / therapeutic use
  • Inositol / analogs & derivatives
  • Inositol / therapeutic use
  • Insulin / therapeutic use
  • Ketosis / diagnosis
  • Ketosis / etiology
  • Male
  • Metformin / therapeutic use
  • Obesity / complications
  • Obesity / metabolism
  • Xanthomatosis / diagnosis*
  • Xanthomatosis / etiology
  • Xanthomatosis / pathology

Substances

  • Acetoacetates
  • Apolipoprotein E2
  • Apolipoprotein E4
  • Glycated Hemoglobin A
  • Hypoglycemic Agents
  • Insulin
  • hemoglobin A1c protein, human
  • Inositol
  • acetoacetic acid
  • Metformin
  • voglibose
  • 3-Hydroxybutyric Acid