Hemochromatosis and Diabetes

Abstract

Hemochromatosis is a rare disorder of metabolism characterized by the deposition throughout the body of abnormally large amounts of hemosiderin and, to a lesser degree, of hemofuscin, particularly in the liver, pancreas, and skin. The three cardinal signs of the disease are hepatomegaly with cirrhosis, diabetes mellitus, and abnormal pigmentation of the skin. In 1865 Trousseau¹ described the first case which seemed to have the characteristic symptoms, although no name was coined by him for this disease. The second case was reported in 1871 by Troisier,² who called the disease “Le cirrhose pigmentaire dans diabète sucré.” Hanot and Chauffard,³ 11 years later, believed that the diabetes associated with hemochromatosis was probably the primary disease and caused cirrhosis as well as pigmentation of the skin. They called the disease “Cirrhose hypertrophique pigmentaire dans le diabète sucré.” Other authors^4–7 similarly considered the hyperglycemia as the primary cause of the disorder and thought that the disturbance of blood sugar homeostasis resulted from the changes in the liver and other organs. Hanot⁵ coined the term “diabète bronzé.” Von Recklinghausen,⁷ in 1889, named the disease hemochromatosis and subsequent investigators^8–13 defined the clinical and pathological entity of this disease.