Dermatologic Manifestations of Localized Lipodystrophy 

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD   more...
 
Updated: May 27, 2011
 

Background

Lipodystrophies are a heterogeneous group of diseases clinically characterized by a congenital or acquired loss of fat in circumscribed, partial, or diffuse areas of the body. As a rule, localized lipodystrophies are not associated with metabolic or systemic disorders, they tend to resolve spontaneously, and they have an excellent prognosis. The main concern is cosmetic when the lesions persist. Controversy exists in their classification, but 3 subsets can be identified based on the presence or the absence of preceding inflammation and the histologic findings.

Centrifugal lipodystrophy or lipodystrophia centrifugalis abdominalis infantilis is the best-characterized subset. It affects children at a young age. It begins on the trunk, spreads for a few years to the neighboring abdomen or chest, and eventually resolves spontaneously in most patients.

The second group is defined by a lack of preceding inflammation and a common histologic pattern of involutional lipoatrophy. It includes such entities as lipoatrophia semicircularis; semicircular or annular dystrophy; and lipoatrophies linked to repeated trauma, pressure, or drug injections. A number of cases are idiopathic.

The third type represents a sequela from various types of panniculitis and is associated with preceding inflammation. It includes lipophagic granulomatous panniculitis and other types of panniculitis, primarily associated with connective-tissue disease.

Other eMedicine articles on lipodystrophy include Generalized Lipodystrophy; HIV Lipodystrophy; and Progressive Lipodystrophy.

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Pathophysiology

The pathophysiology of localized lipodystrophy is unknown. The pathogenetic mechanism of inherited lipodystrophies at the molecular level has been linked to mutations of lamin A/C, peroxisome proliferator-activated receptor (PPAR-gamma), and other seemingly unrelated proteins.[1] In cases linked to injections of insulin, an immune response mediated by tumor necrosis factor-alpha has been hypothesized.

Familial partial lipodystrophy (FPLD) usually results from coding sequence mutations either in LMNA, encoding nuclear lamin A/C, or in peroxisome proliferator-activated receptor-gamma (PPARG), encoding PPAR-gamma. The LMNA form is called FPLD2 (Mendelian Inheritance in Man [MIM] 151660) and the PPARG form is called FPLD3 (MIM 604367). FPLD phenotype may be due to a single-base mutation in the DNA-binding domain of peroxisome PPAR-gamma.[2] Impaired PPARG function through a mutation of a conserved salt bridge (R425C) producing FPLD has also been described.[3]

Segmental lipodystrophy may result from mosaicism, a postzygotic mutation that may be a germline or a somatic phenomenon.[4] Mosaicism of adipocytes may be challenging to classify.

Lipophagic lobular panniculitis, an entity of unknown origin, may lead to circumferential fat atrophy of the ankles and may represent an end-stage manifestation of an idiopathic lobular panniculitis of children localized to the lower part of the lower limbs.[5]

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Epidemiology

Frequency

International

Few cases have been reported internationally. Centrifugal lipodystrophy and the involutional type are rare. Lipoatrophy secondary to panniculitis is more frequent.

Mortality/Morbidity

Localized lipodystrophies usually have little or no associated morbidity or mortality. They are circumscribed and tend to resolve spontaneously, although panniculitis may be chronic and relapsing. Usually, no associated systemic disorder is present, except in some cases of panniculitis induced by an underlying connective-tissue disorder. Even then, symptoms tend to be mild with an excellent overall outcome.

Race

No racial predilection exists except for centrifugal lipodystrophy, which has been described mainly in Asian children.

Sex

No difference in sex has been established, except for a female predominance in the involutional type. A female predominance is also observed in connective-tissue disorder–associated lipoatrophic panniculitis.

Age

No specific age of onset exists except for centrifugal lipodystrophy. In these cases, lesions appear before age 3 years, they stop spreading by age 8 years, and they tend to resolve by age 13 years.

Connective-tissue disorder–associated lipoatrophic panniculitis may be observed at any age, but it occurs most frequently in adults aged 20-60 years. Lupus erythematosus is the most common underlying disease.

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Contributor Information and Disclosures
Author

Robert A Schwartz, MD, MPH  Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

Isabelle Thomas, MD  Associate Professor, Department of Dermatology, University of Medicine and Dentistry of New Jersey, New Jersey Medical School; Chief of Dermatology Service, Veterans Affairs Medical Center of East Orange

Isabelle Thomas, MD is a member of the following medical societies: American Academy of Dermatology and Sigma Xi

Disclosure: Nothing to disclose.

Specialty Editor Board

Daniel Mark Siegel, MD, MS  Director, Procedural Dermatology Fellowship Program, Clinical Professor of Dermatology, Department of Dermatology, State University of New York Downstate

Daniel Mark Siegel, MD, MS is a member of the following medical societies: American Academy of Dermatology, American College of Mohs Micrographic Surgery and Cutaneous Oncology, American College of Physician Executives, American Society for Dermatologic Surgery, American Society for MOHS Surgery, and International Society for Dermatologic Surgery

Disclosure: Nothing to disclose.

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD  Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and American College of Rheumatology

Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Electrical Optical Sciences Consulting fee Consulting; Celgene Honoraria Safety Monitoring Committee; GSK - Glaxo Smith Kline Consulting fee Consulting; TenXBioPharma Consulting fee Safety Monitoring Committee

Glen H Crawford, MD  Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital

Glen H Crawford, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Phi Beta Kappa, and Society of USAF Flight Surgeons

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

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