Progressive Lipodystrophy Treatment & Management
- Author: Robert A Schwartz, MD, MPH; Chief Editor: William D James, MD more...
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.
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. It may be useful in selected patients for the reconstruction of bilateral facial atrophy.
Referral to a nephrologist or an internist may be warranted for progressive lipodystrophy patients with severe nephropathy and those with associated autoimmune diseases.
Hyperalimentation can result in the excessive accumulation of fat in an unaffected area with no improvement on dystrophic areas.
Note the following 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.
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.
Herranz P, de Lucas R, Perez-España L, Mayor M. Lipodystrophy syndromes. Dermatol Clin. 2008 Oct. 26(4):569-78, ix. [Medline].
Kanazawa N. Nakajo-Nishimura Syndrome: An Autoinflammatory Disorder Showing Pernio-Like Rashes and Progressive Partial Lipodystrophy. Allergol Int. 2012 Mar 25. 0(0):[Medline].
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].
Mitchell SW. Singular case of absence of adipose matter in the upper half of the body. Am J Med Sci. 1885. 90:105-106.
Maffeis L, Vercellesi P, Corona F, Forzenigo L, Gelmetti C. Acquired acral lipodystrophy in a 6-year-old girl. Pediatr Dermatol. 2009 Sep-Oct. 26(5):566-8. [Medline].
de Haan W. Lipodystrophy and muscular dystrophy caused by PTRF mutations. Clin Genet. 2010 May. 77(5):436-7. [Medline].
Nabrdalik K, Strózik A, Minkina-Pedras M, Jarosz-Chobot P, Mlynarski W, Grzeszczak W, et al. [Dunnigan-type familial partial lipodystrophy associated with the heterozygous R482W mutation in LMNA gene - case study of three women from one family]. Endokrynol Pol. 2013. 64(4):306-11. [Medline].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
Chong AY, Lupsa BC, Cochran EK, Gorden P. Efficacy of leptin therapy in the different forms of human lipodystrophy. Diabetologia. 2009 Sep 2. [Medline].