We report on a 33-year-old male patient with generalized acquired
lipodystrophy,
insulin resistant
diabetes mellitus and
acanthosis nigricans (Lawrence Syndrome). First probable symptoms of
lipodystrophy (
weight loss, shrinkage of subcutaneous fatty tissue, and loss of muscular strength) became evident three years ago, with the onset of
diabetes mellitus occurring about six months later. The patient suffered from the following clinical symptoms:
IDDM with increasing
insulin-requirement, extreme reduction of fatty tissue,
fatty liver hepatitis with elevated liver
enzymes, glomerulopathy, muscular and
neuropathic pains, as well as hypertriglyceridaemia. A basal
C-peptide concentration is rather high. Definitely, the endogenous insulin secretion is increased. In other words,
insulin resistance is documented. In an effort to identify the pathogenetic mechanisms of
lipoatrophic diabetes mellitus in this patient and to develop a therapeutic strategy,
antibodies against different tissues and endocrinologic regulation were investigated. It was possible to demonstrate the presence of serum
autoantibodies against lipocytes of the subcutis and other tissues, against hepatic stellate cells, together with
autoantibodies against different endocrine organs. By studying the basis of diabetic abnormalities relating to the
growth hormone (GH), the
insulin-like growth factor (IGF) dynamics in this patient, i.e. reductions of GH,
IGF-I,
IGF-II,
IGF-Binding protein (IGF-BP) 2 and IGF-BP 3, were detected. An immunosuppressive treatment strategy was not beneficial.