eMedicine Specialties > Endocrinology > Metabolic Disorders

Lipodystrophy, Acquired Partial: Treatment & Medication

Author: George T Griffing, MD, Professor of Medicine, St Louis University School of Medicine
Coauthor(s): 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
Contributor Information and Disclosures

Updated: Jun 18, 2008

Treatment

Medical Care

  • In general, treatment for acquired partial lipodystrophy is limited to cosmetic, dietary, or medical options.
  • Currently, no effective treatment exists to halt the progression of lipodystrophy.
  • Thiazolidinediones have been used in the management of various types of lipodystrophies. They bind to peroxisome proliferator-activator receptor gamma (PPAR-gamma), which stimulates the transcription of genes responsible for growth and differentiation of adipocytes.16 A single report has suggested a beneficial effect from treatment with rosiglitazone on fat distribution in acquired partial lipodystrophy17 ; however, preferential fat gain was in the lower body.
  • Following the online publication of a meta-analysis,18 the Food and Drug Administration issued an alert on May 21, 2007, to patients and health care professionals warning that rosiglitazone could potentially cause an increased risk of myocardial infarction (MI) and heart-related deaths. A thiazolidinedione derivative, rosiglitazone is an antidiabetic agent that improves glycemic control by improving insulin sensitivity. The drug is highly selective and is a potent agonist for PPAR-gamma. Activation of PPAR-gamma receptors regulates insulin-responsive gene transcription involved in glucose production, transport, and utilization, thereby reducing blood glucose concentrations and hyperinsulinemia. Potent PPAR-gamma agonists have been shown to increase the incidence of edema. A large-scale phase III trial (RECORD) has been underway to study the cardiovascular outcomes of rosiglitazone.
     
    For more information, see the FDA's Safety Alert on Avandia. Additionally, responses to the controversy, including the following articles, can be viewed at Heartwire news (the heart.org, from WebMD): 1) Rosiglitazone increases MI and CV death in meta-analysis, 2) The rosiglitazone aftermath: Legitimate concerns or hype?, and 3) RECORD interim analysis of rosiglitazone safety: No clear-cut answers.
  • Direct drug therapy is administered according to the associated condition.

    • Membranoproliferative glomerulonephritis and the presence of renal dysfunction largely determine the prognosis of acquired partial lipodystrophy. Standard guidelines for the management of renal disease should be followed.
    • The course of membranoproliferative glomerulonephritis in acquired partial lipodystrophy has not been significantly altered by treatment with corticosteroids or cytotoxic medications.
    • Recurrent bacterial infections, if severe, might be managed with prophylactic antibiotics.

Surgical Care

  • The purpose of surgery is mainly cosmetic. According to guidelines from the American Academy of Dermatology, lipodystrophy is one of the indications for fat transplant.
  • Several facial reconstruction techniques have been used, with variable success, to restore facial contour. However, surgical intervention cannot restore adipose tissue distribution in other affected areas.
  • The literature is controversial regarding these procedures. The best approach is to individualize the treatment options based on the patient's condition and requirements. These procedures are not recommended for prepubertal children.
  • Procedures may include the transposition of facial muscles, adipose tissue transplantation (liposuction), and the insertion of silicone or other implants.

Consultations

Early consultation with a nephrologist or an endocrinologist is very important if renal or metabolic complications are suggested.

Diet

No evidence in the literature favors any specific diets in this group of patients. A low-fat, high-carbohydrate diet can be detrimental with regard to triglyceride levels, and weight gain should be avoided to reduce the risk of worsening metabolic status. However, children with this syndrome should be permitted normal food intake to allow for normal growth.

Activity

Regular exercise should be encouraged to help improve metabolic status.

Medication

Pharmacologic intervention is limited in this syndrome.17,18,16 Biguanides and thiazolidinediones have been used in the treatment of the insulin-resistant state (which includes type 2 diabetes and polycystic ovary disease) and in cases of HIV - related glucose intolerance. Although not studied in this group of patients, these drugs should be the first line of treatment if diabetes occurs. Fibrates are the drug of choice for the treatment of hypertriglyceridemia and low HDL cholesterol syndrome.

Hypoglycemic agents

These medications would be started if the patient has developed diabetes that is not being controlled through diet. Insulin sensitizers (biguanides and thiazolidinediones) can be used to reduce insulin levels in women with polycystic ovarian syndrome and with irregular periods.


Metformin (Glucophage)

Reduces hepatic glucose output, decreases intestinal absorption of glucose, and increases glucose uptake in peripheral tissues (muscle and adipocytes). Major drug used in type 2 diabetes and obesity.

Adult

Initial: 500 mg PO bid
Maintenance: 850 mg PO tid

Pediatric

Not established

Diuretics, thyroid products, oral contraceptives, phenytoin, calcium channel blocking drugs, and phenothiazines may decrease effects; cimetidine may increase levels

Documented hypersensitivity; acute myocardial infarction; septicemia; renal disease

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Caution in renal insufficiency; discontinue therapy before performing any surgical procedures; impaired liver function


Pioglitazone (Actos)

Improves target cell response to insulin without increasing insulin secretion from pancreas. Decreases hepatic glucose output and increases insulin-dependent glucose use in skeletal muscle and possibly in liver and adipose tissue.

Adult

15 or 30 mg PO qd; may increase, not to exceed 45 mg/d

Pediatric

Not established

May reduce plasma concentrations of contraceptives containing ethinyl estradiol and norethindrone; lab studies suggest ketoconazole may inhibit metabolism (monitor blood glucose levels closely); in combination with insulin or oral hypoglycemics (eg, sulfonylureas), may increase risk for hypoglycemia

Documented hypersensitivity; active liver disease; ketoacidosis; type 1 diabetes

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Monitor transaminases; discontinue if ALT rises >3 times upper limit of normal; caution in edema and congestive heart failure; may decrease hemoglobin, hematocrit, and white blood cell counts

More on Lipodystrophy, Acquired Partial

Overview: Lipodystrophy, Acquired Partial
Differential Diagnoses & Workup: Lipodystrophy, Acquired Partial
Treatment & Medication: Lipodystrophy, Acquired Partial
Follow-up: Lipodystrophy, Acquired Partial
Multimedia: Lipodystrophy, Acquired Partial
References
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References

  1. Mitchell SW. Singular case of absence of adipose matter in the upper half of the body. Am J Med Sci. 1885;90:105-6.

  2. Misra A, Peethambaram A, Garg A. Clinical features and metabolic and autoimmune derangements in acquired partial lipodystrophy: report of 35 cases and review of the literature. Medicine (Baltimore). Jan 2004;83(1):18-34. [Medline].

  3. Cronin CC, Higgins TJ, Molloy M. Lupus, C3 nephritic factor and partial lipodystrophy. QJM. Apr 1995;88(4):298-9. [Medline].

  4. Walport MJ, Davies KA, Botto M, et al. C3 nephritic factor and SLE: report of four cases and review of the literature. QJM. Oct 1994;87(10):609-15. [Medline].

  5. Walker PD. Dense deposit disease: new insights. Curr Opin Nephrol Hypertens. May 2007;16(3):204-12. [Medline].

  6. Caramaschi P, Biasi D, Lestani M, et al. A case of acquired partial lipodystrophy associated with POEMS syndrome. Rheumatology (Oxford). Mar 2003;42(3):488-90. [Medline]. [Full Text].

  7. Sissons JG, West RJ, Fallows J, et al. The complement abnormalities of lipodystrophy. N Engl J Med. Feb 26 1976;294(9):461-5. [Medline].

  8. Hegele RA, Cao H, Liu DM, et al. Sequencing of the reannotated LMNB2 gene reveals novel mutations in patients with acquired partial lipodystrophy. Am J Hum Genet. Aug 2006;79(2):383-9. [Medline]. [Full Text].

  9. Hegele RA, Joy TR, Al-Attar SA, et al. Thematic review series: adipocyte biology. Lipodystrophies: windows on adipose biology and metabolism. J Lipid Res. Jul 2007;48(7):1433-44. [Medline]. [Full Text].

  10. Demetriou K, Kallikas I, Zouvani I, et al. The pregnant patient with partial lipodystrophy developing acute renal failure--onset of de novo membranoproliferative glomerulonephritis. Nephrol Dial Transplant. Aug 1998;13(8):2121-4. [Medline]. [Full Text].

  11. Meyrier A. The patient with glomerulonephritis and lipodystrophy. Nephrol Dial Transplant. Jan 1997;12(1):226-7. [Medline]. [Full Text].

  12. Chopra S, Isaacs R, Mammen K, et al. Renal transplantation in a patient with Barraquer-Simons disease and mesangiocapillary glomerulonephritis type II. Nephrol Dial Transplant. Oct 2000;15(10):1723-4. [Medline]. [Full Text].

  13. Garg A. Lipodystrophies. Am J Med. Feb 2000;108(2):143-52. [Medline].

  14. Al-Attar SA, Pollex RL, Robinson JF, et al. Quantitative and qualitative differences in subcutaneous adipose tissue stores across lipodystrophy types shown by magnetic resonance imaging. BMC Med Imaging. 2007;7:3. [Medline]. [Full Text].

  15. Pujol RM, Domingo P, Xavier-Matias-Guiu, et al. HIV-1 protease inhibitor-associated partial lipodystrophy: clinicopathologic review of 14 cases. J Am Acad Dermatol. Feb 2000;42(2 Pt 1):193-8. [Medline].

  16. Guettier JM, Park JY, Cochran EK, et al. Leptin therapy for partial lipodystrophy linked to a PPAR-gamma mutation. Clin Endocrinol (Oxf). Apr 2008;68(4):547-54. [Medline].

  17. Walker UA, Kirschfink M, Peter HH. Improvement of acquired partial lipodystrophy with rosiglitazone despite ongoing complement activation. Rheumatology (Oxford). Feb 2003;42(2):393-4. [Medline]. [Full Text].

  18. [Best Evidence] Nissen SE, Wolski K. Effect of rosiglitazone on the risk of myocardial infarction and death from cardiovascular causes. N Engl J Med. Jun 14 2007;356(24):2457-71. [Medline]. [Full Text].

  19. Aragona P, Quattrocchi P, Trombetta CJ, et al. Retinal alterations in acquired partial lipodystrophy: a case report. Arch Ophthalmol. Feb 2002;120(2):218-20. [Medline].

  20. Biasi D, Caramaschi P, Carletto A, et al. A case of acquired partial lipodystrophy associated with localized scleroderma and undifferentiated connective tissue disease. Rheumatol Int. 1999;19(1-2):75-6. [Medline].

  21. Dupéré A, Poulin Y. Facial lipoatrophy following systemic lupus erythematosus. J Cutan Med Surg. May-Jun 2003;7(3):232-5. [Medline].

  22. Haque WA, Shimomura I, Matsuzawa Y, et al. Serum adiponectin and leptin levels in patients with lipodystrophies. J Clin Endocrinol Metab. May 2002;87(5):2395. [Medline]. [Full Text].

  23. Hisamichi K, Suga Y, Hashimoto Y, et al. Two Japanese cases of localized involutional lipoatrophy. Int J Dermatol. Mar 2002;41(3):176-7. [Medline].

  24. Orrell RW, Peatfield RC, Collins CE, et al. Myopathy in acquired partial lipodystrophy. Clin Neurol Neurosurg. May 1995;97(2):181-6. [Medline].

  25. Patel D, Page B. Ocular complications in acquired partial lipodystrophy. Postgrad Med J. Nov 2006;82(973):774. [Medline].

  26. Poley JR, Stickler GB. Progressive lipodystrophy. A clinical study of 50 patients. Am J Dis Child. Oct 1963;106:356-63. [Medline].

  27. Porter WM, O'Gorman-Lalor O, Lane RJ, et al. Barraquer-Simons lipodystrophy, Raynaud's phenomenon and cutaneous vasculitis. Clin Exp Dermatol. Jun 2000;25(4):277-80. [Medline].

  28. Winhoven SM, Hafejee A, Coulson IH. An unusual case of an acquired acral partial lipodystrophy (Barraquer-Simons syndrome) in a patient with extrinsic allergic alveolitis. Clin Exp Dermatol. Jul 2006;31(4):594-6. [Medline].

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Further Reading

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Keywords

Barraquer-Simons syndrome, Barraquer disease, Simons disease, cephalothoracic lipodystrophy, acquired partial lipodystrophy, fat loss, fat hypertrophy, adipocyte lysis, lipohypertrophy, mesangiocapillary glomerulonephritis, membranoproliferative glomerulonephritis, systemic lupus erythematosus, SLE, dermatomyositis, hypothyroidism, pernicious anemia, PA, celiac disease, dermatitis herpetiformis, rheumatoid arthritis, RA, temporal arteritis, leukocytoclastic vasculitis, complement anomaly, complement abnormality, autoimmune process, autoimmune disease

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Contributor Information and Disclosures

Author

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.

Coauthor(s)

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: Pfizer, Novo Nordisk, Merck Honoraria Speaking and teaching

Medical Editor

Amir E Harari, MD, Staff Physician, Endocrinology Division, Instructor, Department of Clinical Medicine, Naval Medical Center at San Diego
Amir E Harari, MD is a member of the following medical societies: Alpha Omega Alpha, American Association of Clinical Endocrinologists, American College of Physicians, and Endocrine Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Arthur B Chausmer, MD, PhD, FACP, FACE, FACN, CNS, 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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