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Acquired Generalized Lipodystrophy

synonym
Lawrence Syndrome

Acquired Generalized Lipodystrophy is a rare disease. It is characterized by generalized disappearance of body fat after birth. These patients have normal fat at birth. 

The onset of lipodystrophy occurs in the childhood and adolescence and may occur following infections such as varicella, measles, pertussis, diphtheria, pneumonia, osteomyelitis, parotitis, infectious mononucleosis, and hepatitis.

Clinical features:
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Patients lose body fat over a period of months or years. Eventually generalized loss of fat may occur resulting in muscular appearance and prominent superficial veins. Almost all areas of the body can be affected although in some patients some areas may be spared.

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Increased linear growth may be seen in the children.

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Dark velvety pigmentation (acanthosis nigricans) may also occur in the axilla and neck.

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Excess body hair, enlargement of genitalia (clitoromegaly) and occasional ovarian cysts may be seen in the females.

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These patients also have elevated basal metabolic rate and a voracious appetite.

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Levels of serum triglycerides are high. Diabetes occurs usually after the onset of lipodystrophy

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Patients with acquired generalized lipodystrophy may also develop other autoimmune disorders like vitiligo (light-colored spots on skin), sicca syndrome, rheumatoid arthritis, dermatomyositis, thyroiditis and chronic active hepatitis.

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There is a female preponderance with a male to female ratio of 1:3, respectively.

 

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