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Progressive Lipodystrophy Workup

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: William D James, MD  more...
 
Updated: Jun 21, 2016
 

Laboratory Studies

Urinalysis may be helpful in progressive lipodystrophy. Approximately one third of patients with this condition have some degree of glomerulonephritis, and, if hypocomplementemia is present, the risk is higher. Manifestations of glomerulonephritis range from asymptomatic proteinuria to a nephrotic syndrome and renal insufficiency.

Baseline creatinine and blood urea nitrogen studies, as well as urine studies, should be performed to detect proteinuria and hematuria.

A CBC count should be obtained. Normochromic normocytic anemia is often present.

Immune studies can be ordered. Immune studies may be needed to identify patients with associated immune disorders, particularly if the diagnosis of partial lipodystrophy is made at a younger age. The presence of serum antinuclear antibodies and anti–double-stranded DNA antibodies has been reported in some patients.

Complement levels may be determined. The most common laboratory abnormality in patients with progressive lipodystrophy is a low complement level. Hypocomplementemia is characterized by a low C3 complement level, a normal C4 level, and the presence of C3 nephritic factor.

Lipid studies may be performed. Hypertriglyceridemia secondary to nephrotic syndrome may be present in some patients.

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Imaging Studies

Head MRIs show evidence of fat loss in progressive lipodystrophy patients.

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Other Tests

Immunofluorescence studies in progressive lipodystrophy patients show deposits of C3 in a granular pattern in the basement membranes.

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Procedures

Skin biopsy findings confirm the progressive lipodystrophy diagnosis. Kidney biopsy is required if a glomerulonephritis is associated with the progressive lipodystrophy.

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Histologic Findings

Cutaneous biopsy in progressive lipodystrophy reveals a reduction or absence of subcutaneous fat in affected areas. Subcutaneous fat cells are decreased in number.

Traces of adipose tissue may be found around hair follicles and sebaceous glands. The dermis and epidermis are normal.

Renal biopsy findings are characterized by a membranoproliferative glomerulonephritis with a proliferation of mesangial cells and matrix, as well as thickening of the basement membranes by amorphous electron-dense deposits.

Immunofluorescence studies show deposits of C3 in a granular pattern in the basement membranes.

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Contributor Information and Disclosures
Author

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

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

Disclosure: Nothing to disclose.

Geover Fernandez, MD, FAAD Staff Physician, Department of Dermatology, Rutgers New Jersey Medical School

Geover Fernandez, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society for MOHS Surgery

Disclosure: Nothing to disclose.

Specialty Editor Board

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

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

Disclosure: Nothing to disclose.

Lester F Libow, MD Dermatopathologist, South Texas Dermatopathology Laboratory

Lester F Libow, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Texas Medical Association

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

David P Fivenson, MD Associate Director, St Joseph Mercy Hospital Dermatology Program, Ann Arbor, Michigan

David P Fivenson, MD is a member of the following medical societies: American Academy of Dermatology, Michigan State Medical Society, Society for Investigative Dermatology, Photomedicine Society, Wound Healing Society, Michigan Dermatological Society, Medical Dermatology Society

Disclosure: Nothing to disclose.

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