Very rare syndrome combining diabetes mellitus and
Troisier-Hanot-Chauffard Syndrome; Leschke Syndrome;
Recklinghausen Applebaum Syndrome; Troisier Syndrome.
Greatest frequency in men
between 40 and 60 years old. The hereditary type is transmitted as an
autosomal recessive trait first described by Troisier in 1871 and by Hanot
and Chauffard in 1882.
Diabetes mellitus associated with hypertrophic
cirrhosis of the liver and dark brownish skin pigmentation caused by
deposition of excess of melanin, iron pigment, or both in tissues.
Lassitude, weakness, weight loss, upper right abdominal quadrant sharp pain,
dyspnea, and loss of libido occur. Occasionally, specific progressive
Assess diabetes and adapt intraoperative
fluid regimen. The insulin management should be based on perioperative
glucose level monitoring. Assess liver function (clinical,
echography, CT, laboratory, including coagulation) and adapt anesthetic
technique and choice of agents in consideration for the potential presence of hepatic toxicity.
Hanot VC, Chauffard AME: Cirrhose hypertrophique pigmentaire dans le
diabète sucré. Rev Med (Paris) 2:385, 1882.
Troisier CE: Diabète sucré. Bull Soc Anat (Paris) 16:231, 1871.