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Lipodystrophy, Generalized: Multimedia

Author: Robert A Gabbay, MD, PhD, Associate Professor of Medicine, Division of Endocrinology, Diabetes and Metabolism, Laurence M Demers Career Development Professor, Penn State College of Medicine; Director, Diabetes Program, Penn State Milton S Hershey Medical Center; Executive Director, Penn State Institute for Diabetes and Obesity
Coauthor(s): Nazia Raja-Khan, MD, Assistant Professor of Medicine, Department of Endocrinology, Penn State University College of Medicine; Elif Arioglu, MD, Assistant Professor of Medicine, Division of Endocrinology and Metabolism, University of Michigan
Contributor Information and Disclosures

Updated: Oct 29, 2009

Multimedia

Clinical spectrum of adiposity. T1-weighted magne...
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Media file 1: Clinical spectrum of adiposity. T1-weighted magnetic resonance images in which lipid appears white. Transverse sections of the torso at the level of the fifth lumbar vertebra (top panels) and of the midthigh (bottom panels). Generalized lipoatrophy (top left, bottom left), a healthy individual with a body mass index of 24 kg/m2 (top center, bottom center), and an individual who is obese with a body mass index of 38 kg/m2 (top right, bottom right). Note the marked absence of all fat depots in the patient with generalized lipoatrophy, with preservation of marrowfat and a lipid-filled liver. In addition, the bright color of the muscle is indicative of lipid-filled myocytes.
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Clinical spectrum of adiposity. T1-weighted magne...

Clinical spectrum of adiposity. T1-weighted magnetic resonance images in which lipid appears white. Transverse sections of the torso at the level of the fifth lumbar vertebra (top panels) and of the midthigh (bottom panels). Generalized lipoatrophy (top left, bottom left), a healthy individual with a body mass index of 24 kg/m2 (top center, bottom center), and an individual who is obese with a body mass index of 38 kg/m2 (top right, bottom right). Note the marked absence of all fat depots in the patient with generalized lipoatrophy, with preservation of marrowfat and a lipid-filled liver. In addition, the bright color of the muscle is indicative of lipid-filled myocytes.

Congenital generalized lipoatrophy in a 16-year-o...
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Media file 2: Congenital generalized lipoatrophy in a 16-year-old girl. In T1-weighted magnetic resonance imaging (MRI) scan, transverse sections of the torso at the fifth lumbar vertebra demonstrate markedly deficient subcutaneous and visceral fat (top left). Transverse sections of the midthigh demonstrate an absence of subcutaneous fat, deep fat, intrafascicular fat, and marrow fat (bottom left); note light-colored, lipid-filled muscle tissue. Note absence of fat throughout the body (right).
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Congenital generalized lipoatrophy in a 16-year-o...

Congenital generalized lipoatrophy in a 16-year-old girl. In T1-weighted magnetic resonance imaging (MRI) scan, transverse sections of the torso at the fifth lumbar vertebra demonstrate markedly deficient subcutaneous and visceral fat (top left). Transverse sections of the midthigh demonstrate an absence of subcutaneous fat, deep fat, intrafascicular fat, and marrow fat (bottom left); note light-colored, lipid-filled muscle tissue. Note absence of fat throughout the body (right).

Dunnigan familial partial lipodystrophy in a 26-y...
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Media file 3: Dunnigan familial partial lipodystrophy in a 26-year-old woman. Note increased fat around the face/neck (top left) and deficiency in the gluteal region and lower extremities (top right). T1-weighted magnetic resonance imaging (MRI) scan of transverse sections of the torso at the fifth lumbar vertebra demonstrate markedly deficient subcutaneous fat and increased visceral fat (bottom left). Transverse sections of the midthigh demonstrate absence of subcutaneous fat, with preservation of deep fat, intrafascicular fat, and marrow fat (bottom right).
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Dunnigan familial partial lipodystrophy in a 26-y...

Dunnigan familial partial lipodystrophy in a 26-year-old woman. Note increased fat around the face/neck (top left) and deficiency in the gluteal region and lower extremities (top right). T1-weighted magnetic resonance imaging (MRI) scan of transverse sections of the torso at the fifth lumbar vertebra demonstrate markedly deficient subcutaneous fat and increased visceral fat (bottom left). Transverse sections of the midthigh demonstrate absence of subcutaneous fat, with preservation of deep fat, intrafascicular fat, and marrow fat (bottom right).

Werner syndrome in a 37-year-old woman (left). Cl...
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Media file 4: Werner syndrome in a 37-year-old woman (left). Clinical features evident include premature aging, alopecia, sclerodermalike changes in the skin, and peripheral lipoatrophy (shown). Insulin-resistant diabetes is an associated feature. On the right is mandibuloacral dysplasia in a 14-year-old girl. This rare recessive syndrome is characterized by acroosteolysis, widened sutures, and hypoplastic mandible (shown) and clavicles (shown). Marked peripheral lipoatrophy and insulin resistance are associated features.
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Werner syndrome in a 37-year-old woman (left). Cl...

Werner syndrome in a 37-year-old woman (left). Clinical features evident include premature aging, alopecia, sclerodermalike changes in the skin, and peripheral lipoatrophy (shown). Insulin-resistant diabetes is an associated feature. On the right is mandibuloacral dysplasia in a 14-year-old girl. This rare recessive syndrome is characterized by acroosteolysis, widened sutures, and hypoplastic mandible (shown) and clavicles (shown). Marked peripheral lipoatrophy and insulin resistance are associated features.

Acquired generalized lipoatrophy is in the differ...
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Media file 5: Acquired generalized lipoatrophy is in the differential diagnosis of congenital generalized lipoatrophy. This patient, who had completely normal fat distribution at age 3 years 8 months (left), acutely lost her body fat with a febrile illness at age 6 years 10 months (right).
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Acquired generalized lipoatrophy is in the differ...

Acquired generalized lipoatrophy is in the differential diagnosis of congenital generalized lipoatrophy. This patient, who had completely normal fat distribution at age 3 years 8 months (left), acutely lost her body fat with a febrile illness at age 6 years 10 months (right).

Loss of muscle and fat tissue in a 19-year-old wo...
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Media file 6: Loss of muscle and fat tissue in a 19-year-old woman with severe juvenile dermatomyositis and generalized lipodystrophy. T1-weighted magnetic resonance imaging (MRI) scan of transverse sections of the patient's torso at the fifth lumbar vertebra demonstrate markedly deficient subcutaneous fat and markedly increased visceral fat (top left). Transverse sections of the midthigh demonstrate asymmetrical absence of subcutaneous fat, with preservation of medial subcutaneous fat and marrow fat (bottom left).
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Loss of muscle and fat tissue in a 19-year-old wo...

Loss of muscle and fat tissue in a 19-year-old woman with severe juvenile dermatomyositis and generalized lipodystrophy. T1-weighted magnetic resonance imaging (MRI) scan of transverse sections of the patient's torso at the fifth lumbar vertebra demonstrate markedly deficient subcutaneous fat and markedly increased visceral fat (top left). Transverse sections of the midthigh demonstrate asymmetrical absence of subcutaneous fat, with preservation of medial subcutaneous fat and marrow fat (bottom left).

More on Lipodystrophy, Generalized

References
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Further Reading

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Keywords

generalized lipodystrophy, lipodystrophy, lipoatrophy, SHORT syndrome, adipose tissue, Werner syndrome, Werner's syndrome, atrophic lesions, tissue atrophy, lipoatrophic, fat lesions, fat lesion, Emery-Dreifuss muscular dystrophy

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Contributor Information and Disclosures

Author

Robert A Gabbay, MD, PhD, Associate Professor of Medicine, Division of Endocrinology, Diabetes and Metabolism, Laurence M Demers Career Development Professor, Penn State College of Medicine; Director, Diabetes Program, Penn State Milton S Hershey Medical Center; Executive Director, Penn State Institute for Diabetes and Obesity
Robert A Gabbay, MD, PhD is a member of the following medical societies: American Association of Clinical Endocrinologists, American Diabetes Association, and Endocrine Society
Disclosure: Novo Nordisk Honoraria Speaking and teaching; Merck Honoraria Speaking and teaching

Coauthor(s)

Nazia Raja-Khan, MD, Assistant Professor of Medicine, Department of Endocrinology, Penn State University College of Medicine
Nazia Raja-Khan, MD is a member of the following medical societies: American Association of Clinical Endocrinologists, American Diabetes Association, and Endocrine Society
Disclosure: Nothing to disclose.

Elif Arioglu, MD, Assistant Professor of Medicine, Division of Endocrinology and Metabolism, University of Michigan
Elif Arioglu, MD is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine, American Diabetes Association, American Medical Association, and Endocrine Society
Disclosure: Nothing to disclose.

Medical Editor

David M Klachko, MBBCh, Professor Emeritus, Department of Internal Medicine, Division of Endocrinology, Diabetes and Metabolism, University of Missouri
David M Klachko, MBBCh is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, American Diabetes Association, American Federation for Medical Research, Endocrine Society, Missouri State Medical Association, and Sigma Xi
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Arthur B Chausmer, MD, PhD, FACP, FACE, FACN, CNS, Professor of Medicine (Endocrinology, Adj), Johns Hopkins School of Medicine; Affiliate Research Professor, Bioinformatics and Computational Biology Program, School of Computational Sciences, George Mason University; Principal, C/A Informatics, LLC
Arthur B Chausmer, MD, PhD, FACP, FACE, FACN, CNS is a member of the following medical societies: American Association of Clinical Endocrinologists, American College of Endocrinology, American College of Nutrition, American College of Physician Executives, American College of Physicians, American College of Physicians-American Society of Internal Medicine, American Medical Informatics Association, American Society for Bone and Mineral Research, American Society of Law Medicine and Ethics, Endocrine Society, and International Society for Clinical Densitometry
Disclosure: Nothing to disclose.

CME Editor

Mark Cooper, MBBS, PhD, FRACP, Head, Diabetes & Metabolism Division, Baker Heart Research Institute, Professor of Medicine, Monash University
Disclosure: Nothing to disclose.

Chief Editor

George T Griffing, MD, Professor of Medicine, St Louis University School of Medicine
George T Griffing, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Medical Practice Executives, American College of Physician Executives, American College of Physicians, American Diabetes Association, American Federation for Medical Research, American Heart Association, Central Society for Clinical Research, Endocrine Society, International Society for Clinical Densitometry, and Southern Society for Clinical Investigation
Disclosure: Nothing to disclose.

 
 
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