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Previous Volume 350:1220-1234 March 18, 2004 Number 12 Next

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Lipodystrophies are clinically heterogeneous acquired or inherited disorders characterized by the selective loss of adipose tissue. Affected patients are predisposed to insulin resistance and its attendant complications, including diabetes mellitus, dyslipidemia, hepatic steatosis, and acanthosis nigricans. Features of polycystic ovary syndrome — hirsutism, oligoamenorrhea, and polycystic ovaries — may develop in affected women. The mechanisms involved in the pathogenesis of various types of lipodystrophy are listed in Table 1.

View this table: [in this window] [in a new window]   Table 1. Clinical Features and Pathogenetic Basis of Various Types of Lipodystrophies.

 
More than a century after the clinical phenotype was first described,¹ we are beginning to understand the molecular and cellular . . . [Full Text of this Article]

Acquired Lipodystrophies

Lipodystrophy in Patients with Human Immunodeficiency Virus Infection

            Clinical Picture

            Pathogenetic Basis

Acquired Partial Lipodystrophy (The Barraquer–Simons Syndrome)

            Clinical Picture

            Pathogenetic Basis

Acquired Generalized Lipodystrophy

            Clinical Picture

            Pathogenetic Basis

Localized Lipodystrophies

Inherited Lipodystrophies

Congenital Generalized Lipodystrophy (The Berardinelli–Seip Syndrome)

            Clinical Picture

            Molecular Basis of Congenital Generalized Lipodystrophy Type 1

            Molecular Basis of Congenital Generalized Lipodystrophy Type 2

            Other Types of Congenital Generalized Lipodystrophy

Dunnigan Variety of Familial Partial Lipodystrophy

            Clinical Picture

            Molecular Basis

Familial Partial Lipodystrophy Associated with PPAR Gene Mutations

Other Types of Familial Partial Lipodystrophy

Lipodystrophy Associated with Mandibuloacral Dysplasia

            Clinical Picture

            Molecular Basis

Other Inherited Lipodystrophies

Mechanisms of Insulin Resistance and Metabolic Complications

Therapeutic Approaches

Improving Cosmetic Appearance

Management of Dyslipidemia

Management of Hyperglycemia

Additional Considerations for HIV-Infected Patients with Lipodystrophy

Other Therapeutic Options

Conclusions

Source Information

From the Division of Nutrition and Metabolic Diseases, Department of Internal Medicine, and the Center for Human Nutrition, University of Texas Southwestern Medical Center, Dallas.

Address reprint requests to Dr. Garg at the Center for Human Nutrition, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd., Dallas, TX 75390-9052, or at abhimanyu.garg@utsouthwestern.edu.

Related Letters:

Acquired and Inherited Lipodystrophies
Walker U. A., Schott M., Scherbaum W. A., Bornstein S. R., Garg A.
Extract | Full Text | PDF  
N Engl J Med 2004; 351:103-104, Jul 1, 2004. Correspondence

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