Dermatologic Manifestations of Generalized Lipodystrophy Treatment & Management

  • Author: Camila K Janniger, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: May 27, 2011
 

Medical Care

Successful treatment of acanthosis nigricans (AN) in association with generalized lipodystrophy was accomplished in 1 patient with etretinate beginning at 75 mg/d.[6] Therapy for AN was undertaken in this patient for cosmetic reasons. After 4 weeks, the cutaneous eruption had disappeared, with only modest elevation of the preexisting hyperlipemia.

Substitution of eucaloric medium-chain triglycerides for long-chain fatty acids in the diet of 1 patient led to improvement of chylomicronemia, xanthomatosis, hypertriglyceridemia, hepatomegaly, carbohydrate tolerance, and hyperinsulinemia, but not lipoatrophy. Dietary fish oil also may be useful in improving AN.

Because conventional lipid-lowering and antihyperglycemic medications may be insufficient to control severe metabolic abnormalities, leptin has been used for severe lipodystrophy and found to significantly improve metabolic abnormalities.[7] This new option for therapy is leptin, an adipocyte hormone, which may improve insulin resistance, hyperglycemia, dyslipidemia, and hepatic steatosis.[8] Leptin is the first of a group of adipocyte-secreted hormones to be used clinically.[9] Its long-term efficacy in 15 patients with generalized lipodystrophy was evaluated.[10]

Leptin has been judged the first novel, effective, long-term treatment for severe forms of lipodystrophy. Treating severe lipodystrophy as a leptin deficiency syndrome has been shown to improve the metabolic outcomes in those affected patients.[11] Metreleptin treatment has been well tolerated, with typical daily replacement doses of metreleptin at 0.06-0.08 mg/kg for female patients and 0.04mg/kg for male patients, administered by subcutaneous injection twice daily. However, metreleptin is not yet approved for routine clinical use.

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Surgical Care

Surgical intervention may be helpful for patients with deformities.

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Consultations

Consult specialists to manage the variety of medical problems that can occur.

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Diet

The limited ability to store energy as fat means patients with generalized lipodystrophy must maintain a rigid special diet with 4 regular-sized meals each day.

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Contributor Information and Disclosures
Author

Camila K Janniger, MD  Clinical Professor of Dermatology, Clinical Associate Professor of Pediatrics, Chief of Pediatric Dermatology, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Camila K Janniger, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Coauthor(s)

Cris Jagar, MD  Staff Physician, Department of Psychiatry, Trinitas Regional Medical Center

Disclosure: Nothing to disclose.

Specialty Editor Board

Albert C Yan, MD  Section Chief, Associate Professor, Department of Pediatrics, Section of Dermatology, Children's Hospital of Philadelphia and University of Pennsylvania School of Medicine

Albert C Yan, MD is a member of the following medical societies: American Academy of Dermatology, American Academy of Pediatrics, Society for Investigative Dermatology, and Society for Pediatric Dermatology

Disclosure: Nothing to disclose.

Michael J Wells, MD  Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

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.

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.

References

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