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Progressive Lipodystrophy Treatment & Management

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

Medical Care

No specific treatment for progressive lipodystrophy is effective. Symptomatic therapy should be prescribed as necessary for the treatment of renal complications and associated autoimmune disorders in progressive lipodystrophy patients.

Renal and immunologic disturbances may warrant inpatient care at times.

During pregnancy, the health status of the fetus should be ascertained with modalities such as an electronic fetal monitor. Fetal health assessment is particularly important during the third trimester to reduce the risk of intrauterine fetal death associated with progressive lipodystrophy.

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Surgical Care

Various surgical techniques have been adopted through the years in an effort to improve progressive lipodystrophy patients’ facial appearance.[19, 20] Dermal-fat grafts from the gluteal region, temporal muscle flaps, silicone-filling material, and subcutaneous injections of fat from unaffected areas have been used with variable results. A novel surgical technique consists of a 1-stage transfer of 2 paddles of thoracodorsal artery perforator flap with 1 pair of vascular anastomoses for simultaneous restoration of bilateral facial atrophy.[3] It may be useful in selected patients for the reconstruction of bilateral facial atrophy.

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Consultations

Referral to a nephrologist or an internist may be warranted for progressive lipodystrophy patients with severe nephropathy and those with associated autoimmune diseases.

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Diet

Hyperalimentation can result in the excessive accumulation of fat in an unaffected area with no improvement on dystrophic areas.

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Complications

Note the following complications:

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Long-Term Monitoring

Note the following:

  • Renal status: Patients should be monitored regularly for evidence of glomerulonephritis. Glomerulonephritis can develop more than 10 years after the onset of progressive lipodystrophy.
  • Autoimmune disorders: Patients should be monitored for the development of systemic lupus erythematosus because it was reported in a few patients 2-28 years after the onset of progressive lipodystrophy. [22] Other autoimmune disorders have also been associated with this disease.
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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.

References
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  2. Kanazawa N. Nakajo-Nishimura Syndrome: An Autoinflammatory Disorder Showing Pernio-Like Rashes and Progressive Partial Lipodystrophy. Allergol Int. 2012 Mar 25. 0(0):[Medline].

  3. Okazaki M, Tanaka K, Kodaira S, Homma T, Miyashita H. One-Stage Transfer of 2 Paddles of Thoracodorsal Artery Perforator Flap With 1 Pair of Vascular Anastomoses for Barraquer-Simons Syndrome. J Craniofac Surg. 2012 May 4. [Medline].

  4. Mitchell SW. Singular case of absence of adipose matter in the upper half of the body. Am J Med Sci. 1885. 90:105-106.

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  8. Krawiec P, Mełges B, Pac-Kożuchowska E, Mroczkowska-Juchkiewicz A, Czerska K. Fitting the pieces of the puzzle together: a case report of the Dunnigan-type of familial partial lipodystrophy in the adolescent girl. BMC Pediatr. 2016 Mar 14. 16:38. [Medline].

  9. Liu L, Jiang Q, Wang X, Zhang Y, Lin RC, Lam SM, et al. Adipose-specific knockout of SEIPIN/BSCL2 results in progressive lipodystrophy. Diabetes. 2014 Jul. 63(7):2320-31. [Medline].

  10. Ferrarini A, Milani D, Bottigelli M, Cagnoli G, Selicorni A. Two new cases of Barraquer-Simons syndrome. Am J Med Genet A. 2004 May 1. 126A(4):427-9. [Medline].

  11. Haxton MJ. Progressive partial lipodystrophy in association with intrauterine death and growth retardation. Am J Obstet Gynecol. 1983 Dec 1. 147(7):837-8. [Medline].

  12. Fukumoto D, Kubo Y, Saito M, Arase S. Centrifugal lipodystrophy of the scalp presenting with an arch-form alopecia: a 10-year follow-up observation. J Dermatol. 2009 Sep. 36(9):499-503. [Medline].

  13. Payapvipapong K, Niumpradit N, Nakakes A, Buranawuti K. A rare case of acquired partial lipodystrophy (Barraquer-Simons syndrome) with localized scleroderma. Int J Dermatol. 2014 Jan. 53(1):82-4. [Medline].

  14. Kurugol Z, Ulger Z, Berk O, Tugral O. Acquired partial lipodystrophy associated with varicella. Turk J Pediatr. 2009 Nov-Dec. 51(6):617-20. [Medline].

  15. Spranger S, Spranger M, Tasman AJ, Reith W, Voigtlander T, Voigtlander V. Barraquer-Simons syndrome (with sensorineural deafness): a contribution to the differential diagnosis of lipodystrophy syndromes. Am J Med Genet. 1997 Sep 5. 71(4):397-400. [Medline].

  16. Hagari Y, Sasaoka R, Nishiura S, Ishihara M, Mihara M, Shimao S. Centrifugal lipodystrophy of the face mimicking progressive lipodystrophy. Br J Dermatol. 1992 Oct. 127(4):407-10. [Medline].

  17. Sorkina E, Frolova E, Rusinova D, Polyakova S, Roslavtseva E, Vasilyev E, et al. Progressive Generalized Lipodystrophy as a Manifestation of Autoimmune Polyglandular Syndrome Type 1. J Clin Endocrinol Metab. 2016 Apr. 101 (4):1344-7. [Medline].

  18. Kumar NS, Shashibhushan J, Malappa, Venugopal K, Vishwanatha H, Menon M. Lipodystrophy in Human Immunodeficiency Virus (HIV) Patients on Highly Active Antiretroviral Therapy (HAART). J Clin Diagn Res. 2015 Jul. 9 (7):OC05-8. [Medline].

  19. Coessens BC, Van Geertruyden JP. Simultaneous bilateral facial reconstruction of a Barraquer-Simons lipodystrophy with free TRAM flaps. Plast Reconstr Surg. 1995 Apr. 95(5):911-5. [Medline].

  20. Serra JM, Ballesteros A, Mesa F, Bazan A, Paloma V, Sanz J. Use of the temporal muscle flap in Barraquer-Simon's progressive lipodystrophy. Ann Plast Surg. 1993 Feb. 30(2):180-2. [Medline].

  21. Quecedo E, Febrer I, Serrano G, Martinez-Aparicio A, Aliaga A. Partial lipodystrophy associated with juvenile dermatomyositis: report of two cases. Pediatr Dermatol. 1996 Nov-Dec. 13(6):477-82. [Medline].

  22. Font J, Herrero C, Bosch X, Cervera R, Ingelmo M, Mascaro JM. Systemic lupus erythematosus in a patient with partial lipodystrophy. J Am Acad Dermatol. 1990 Feb. 22(2 Pt 2):337-40. [Medline].

  23. Chong AY, Lupsa BC, Cochran EK, Gorden P. Efficacy of leptin therapy in the different forms of human lipodystrophy. Diabetologia. 2009 Sep 2. [Medline].

 
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