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Acquired generalized lipodystrophy

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Alternate names

Lawrence syndrome; Lawrence-Seip syndrome; Acquired lipoatrophic diabetes

Definition

A rare lipodystrophic syndrome characterized by loss of adipose tissue, and is a syndrome of insulin resistance that leads to increased cardiovascular risk. Acquired generalized lipodystrophy is related to a selective loss of subcutaneous adipose tissue occurring exclusively at the extremities (face, legs, arms, palms and sometimes soles).

Epidemiology

More than 100 cases have been described and the female to male ratio is 3:1.

Cause

  • The cause of the disease remains unknown.
  • There may be infectious triggering factors (there was a recent case of the panniculitis type that appeared after tuberculosis) or an autoimmune mechanism.
  • A recent publication showed activation of the classical complement pathway (low C4). This is in contrast to acquired partial lipodystrophy which affects the upper half of the body and is characterized by an activation of the alternative complement pathway (low C3).
  • Progression towards partial lipoatrophy, focal or generalized, has been reported in patients with dermatomyositis, amongst whom this could be a late relapse, and it is more common that the antibody anti-p155 is present.
  • The hypothesis of an underlying genetic factor has not been rejected.

Signs and symptoms

Clinical presentation

For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. 100% of people have these symptoms

80%-99% of people have these symptoms

30%-79% of people have these symptoms

  • Autoimmunity(Autoimmune disease)
  • Calf muscle pseudohypertrophy
  • Cardiomyopathy(Disease of the heart muscle)
  • Hepatic steatosis(Fatty infiltration of liver)
  • Insulin-resistant diabetes mellitus(Insulin resistant diabetes)
  • Progeroid facial appearance(Premature aged appearance)

5%-29% of people have these symptoms

1%-4% of people have these symptoms

Diagnosis

Diagnosis is clinical and should be confirmed by an assessment of body fat, in particular by biphotonic absorptiometry and magnetic resonance imaging.

Treatment

  • The treatment of the metabolic manifestations is a priori no different to the treatment of other forms of insulin resistance: physical exercise, insulin sensitizers such as metformin or pioglitazone, insulin (or preferably insulin analogues), antihypertensives, and monitoring and treatment of hypertriglyceridemia.
  • The efficacy of recombinant human leptin has been demonstrated on the metabolic level but this therapy is not available in all countries.
  • In serious autoimmune forms of the disease, immunosuppressive therapy may be indicated.

Prognosis

The prognosis is not well known but is probably related to cardiovascular risk (linked to the insulin-resistance syndrome) and to the underlying cause of the disease.

NIH genetic and rare disease info

Acquired generalized lipodystrophy is a rare disease.

Latest research - Acquired generalized lipodystrophy

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