eMedicine Specialties > Dermatology > Diseases of the Subcutaneous Tissue

Lipodystrophy, Progressive: Follow-up

Author: Geover Fernandez, MD, FAAD, Staff Physician, Department of Dermatology, University of Medicine and Dentistry New Jersey, New Jersey Medical School
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
Contributor Information and Disclosures

Updated: Mar 28, 2007

Follow-up

Further Inpatient Care

  • 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.

Further Outpatient Care

  • 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.
  • Other autoimmune disorders have also been associated with this disease.

Inpatient & Outpatient Medications

  • No inpatient or outpatient medications are specific for lipodystrophy.
  • Symptomatic therapy may be required for patients with nephropathy or associated immune disorders.

Complications

  • Renal disease
    • Mesangioproliferative glomerulonephritis occurs in 50% of patients with lipodystrophy and a low C3 complement level.
    • Mesangioproliferative glomerulonephritis often follows an aggressive course and may lead to renal insufficiency.
  • Immune disorders
  • Systemic lupus may develop in a few patients.
  • Other autoimmune diseases include dermatomyositis, rheumatoid arthritis, leukocytoclastic vasculitis, hypothyroidism, and pernicious anemia.

Prognosis

  • The prognosis is correlated with the renal complications and the onset of renal failure.

Miscellaneous

Medicolegal Pitfalls

  • Failure to monitor for glomerulonephritis and its complications
  • Failure to closely monitor a high-risk pregnancy

Special Concerns

  • Underlying renal disease
  • Associated autoimmune disorder in some cases
  • High-risk pregnancy with a risk of fetal death
 


More on Lipodystrophy, Progressive

Overview: Lipodystrophy, Progressive
Differential Diagnoses & Workup: Lipodystrophy, Progressive
Treatment & Medication: Lipodystrophy, Progressive
Follow-up: Lipodystrophy, Progressive
References
[ CLOSE WINDOW ]

References

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

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

  3. Fitch N, Tulandi T. Progressive partial lipodystrophy and third-trimester intrauterine fetal death. Am J Obstet Gynecol. May 1987;156(5):1195-6. [Medline].

  4. Font J, Herrero C, Bosch X, et al. Systemic lupus erythematosus in a patient with partial lipodystrophy. J Am Acad Dermatol. Feb 1990;22(2 Pt 2):337-40. [Medline].

  5. Greene AK. Lluis Barraquer-Roviralta (1855-1928): Spanish neurologist described progressive lipodystrophy. Plast Reconstr Surg. Jan 2001;107(1):158-62. [Medline].

  6. Hagari Y, Sasaoka R, Nishiura S, et al. Centrifugal lipodystrophy of the face mimicking progressive lipodystrophy. Br J Dermatol. Oct 1992;127(4):407-10. [Medline].

  7. Halazonetis J. A case of progressive lipodystrophy. Barraquer-Simon''s disease. Br J Oral Surg. Nov 1968;6(2):130-3. [Medline].

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

  9. 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.

  10. Ketterings C. Lipodystrophy and its treatment. Ann Plast Surg. Dec 1988;21(6):536-43. [Medline].

  11. Levy Y, George J, Yona E, Shoenfeld Y. Partial lipodystrophy, mesangiocapillary glomerulonephritis, and complement dysregulation. An autoimmune phenomenon. Immunol Res. Aug 1998;18(1):55-60. [Medline].

  12. Mathieson PW, Wurzner R, Oliveria DB, et al. Complement-mediated adipocyte lysis by nephritic factor sera. J Exp Med. Jun 1 1993;177(6):1827-31. [Medline].

  13. McNeill AD. Progressive lipodystrophy. Br J Surg. Mar 1966;53(3):216-8. [Medline].

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

  15. Perrot H, Delaup JP, Chouvet B. [Barraquer and Simons lipodystrophy. Complement anomalies and cutaneous leukocytoclasic vasculitis]. Ann Dermatol Venereol. 1987;114(9):1083-91. [Medline].

  16. Polsky B, Kotler D, Steinhart C. HIV-associated wasting in the HAART era: guidelines for assessment, diagnosis, and treatment. AIDS Patient Care STDS. Aug 2001;15(8):411-23. [Medline].

  17. Pope E, Janson A, Khambalia A, Feldman B. Childhood acquired lipodystrophy: a retrospective study. J Am Acad Dermatol. Dec 2006;55(6):947-50. [Medline].

  18. Quecedo E, Febrer I, Serrano G, et al. Partial lipodystrophy associated with juvenile dermatomyositis: report of two cases. Pediatr Dermatol. Nov-Dec 1996;13(6):477-82. [Medline].

  19. Requena Caballero C, Angel Navarro Mira M, Bosch IF. Barraquer-Simons lipodystrophy associated with antiphospholipid syndrome. J Am Acad Dermatol. Oct 2003;49(4):768-9. [Medline].

  20. Rifkind BM, Boyle JA, Gale M. Blood lipid levels, thyroid status, and glucose tolerance in progressive partial lipodystrophy. J Clin Pathol. Jan 1967;20(1):52-5. [Medline].

  21. Serra JM, Ballesteros A, Mesa F, et al. Use of the temporal muscle flap in Barraquer-Simon''s progressive lipodystrophy. Ann Plast Surg. Feb 1993;30(2):180-2. [Medline].

  22. 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].

  23. Smak Gregoor PJ, van Bommel EF, Ketterings C, et al. Progressive lipodystrophy, a diagnosis at a glance. Nephrol Dial Transplant. Feb 1998;13(2):507-9. [Medline].

  24. Sonnino M, Ribuffo D, Piovano L, et al. [Nonprogressive, late-onset atrophy of the cheek]. Minerva Chir. Dec 2000;55(12):881-5. [Medline].

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

  26. Van Etten E, Branisteanu DD, Overbergh L, et al. Combination of a 1,25-dihydroxyvitamin D3 analog and a bisphosphonate prevents experimental autoimmune encephalomyelitis and preserves bone. Bone. Apr 2003;32(4):397-404. [Medline].

  27. 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].

  28. Williams DG. C3 nephritic factor and mesangiocapillary glomerulonephritis. Pediatr Nephrol. Feb 1997;11(1):96-8. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

progressive partial lipodystrophy, Barraquer-Simons syndrome, acquired partial lipodystrophy, cephalothoracic dystrophy, acquired progressive lipodystrophy, Kobberling-Dunnigan syndrome, familial mandibuloacral dysplasia syndrome, generalized lipodystrophy, metabolic anomalies, glucose intolerance, hypertriglyceridemia, hypocomplementemia, glomerulonephritis, autoimmune disorders

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Geover Fernandez, MD, FAAD, Staff Physician, Department of Dermatology, University of Medicine and Dentistry New Jersey, New Jersey Medical School
Geover Fernandez, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and American Society for MOHS Surgery
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.

Medical Editor

David P Fivenson, MD, Director, Wound Care Service, Department of Dermatology, Henry Ford Health System
David P Fivenson, MD is a member of the following medical societies: American Academy of Dermatology, Medical Dermatology Society, Michigan Dermatological Society, Michigan State Medical Society, Photomedicine Society, Society for Investigative Dermatology, and Wound Healing Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
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.

Managing Editor

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, European Academy of Dermatology and Venereology, International Society of Dermatology, Massachusetts Medical Society, New York Academy of Sciences, Phi Beta Kappa, Society for Investigative Dermatology, and Texas Medical Association
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System
William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology
Disclosure: elsevier Royalty Other; american college of physicians Honoraria Other

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.