Progressive Lipodystrophy Follow-up
- Author: Robert A Schwartz, MD, MPH; Chief Editor: William D James, MD more...
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
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.[14] 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
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.
- Immune disorders: Systemic lupus may develop in a few patients. Other autoimmune diseases include dermatomyositis,[15]rheumatoid arthritis, leukocytoclastic vasculitis, hypothyroidism, and pernicious anemia.
Prognosis
The prognosis for progressive lipodystrophy is correlated with the renal complications and the onset of renal failure.
Yavuz S, Acarturk TO. Acquired partial lipodystrophy with C3 hypocomplementemia and antiphospholipid and anticardiolipin antibodies. Pediatr Dermatol. Sep-Oct 2010;27(5):504-8. [Medline].
Herranz P, de Lucas R, Perez-España L, Mayor M. Lipodystrophy syndromes. Dermatol Clin. Oct 2008;26(4):569-78, ix. [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. Sep-Oct 2009;26(5):566-8. [Medline].
de Haan W. Lipodystrophy and muscular dystrophy caused by PTRF mutations. Clin Genet. May 2010;77(5):436-7. [Medline].
Ferrarini A, Milani D, Bottigelli M, Cagnoli G, Selicorni A. Two new cases of Barraquer-Simons syndrome. Am J Med Genet A. May 1 2004;126A(4):427-9. [Medline].
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].
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. Sep 2009;36(9):499-503. [Medline].
Kurugol Z, Ulger Z, Berk O, Tugral O. Acquired partial lipodystrophy associated with varicella. Turk J Pediatr. Nov-Dec 2009;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. Sep 5 1997;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. Oct 1992;127(4):407-10. [Medline].
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].
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. Feb 1993;30(2):180-2. [Medline].
Chong AY, Lupsa BC, Cochran EK, Gorden P. Efficacy of leptin therapy in the different forms of human lipodystrophy. Diabetologia. Sep 2 2009;[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. Feb 1990;22(2 Pt 2):337-40. [Medline].
Quecedo E, Febrer I, Serrano G, Martinez-Aparicio A, Aliaga A. Partial lipodystrophy associated with juvenile dermatomyositis: report of two cases. Pediatr Dermatol. Nov-Dec 1996;13(6):477-82. [Medline].
Fitch N, Tulandi T. Progressive partial lipodystrophy and third-trimester intrauterine fetal death. Am J Obstet Gynecol. May 1987;156(5):1195-6. [Medline].
Greene AK. Lluis Barraquer-Roviralta (1855-1928): Spanish neurologist described progressive lipodystrophy. Plast Reconstr Surg. Jan 2001;107(1):158-62. [Medline].
Halazonetis J. A case of progressive lipodystrophy. Barraquer-Simon's disease. Br J Oral Surg. Nov 1968;6(2):130-3. [Medline].
Hegele RA, Cao H, Liu DM, Costain GA, Charlton-Menys V, Rodger NW. 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].
Ketterings C. Lipodystrophy and its treatment. Ann Plast Surg. Dec 1988;21(6):536-43. [Medline].
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].
Mathieson PW, Wurzner R, Oliveria DB, Lachmann PJ, Peters DK. Complement-mediated adipocyte lysis by nephritic factor sera. J Exp Med. Jun 1 1993;177(6):1827-31. [Medline].
McNeill AD. Progressive lipodystrophy. Br J Surg. Mar 1966;53(3):216-8. [Medline].
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].
[Guideline] 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].
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].
Requena Caballero C, Angel Navarro Mira M, Bosch IF, Bauxauli JM, Aliaga Boniche A. Barraquer-Simons lipodystrophy associated with antiphospholipid syndrome. J Am Acad Dermatol. Oct 2003;49(4):768-9. [Medline].
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].
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].
Smak Gregoor PJ, van Bommel EF, Ketterings C, van Saase JL, Kramer P. Progressive lipodystrophy, a diagnosis at a glance. Nephrol Dial Transplant. Feb 1998;13(2):507-9. [Medline].
Sonnino M, Ribuffo D, Piovano L, Cigna E, Scuderi N. [Nonprogressive, late-onset atrophy of the cheek]. Minerva Chir. Dec 2000;55(12):881-5. [Medline].
Van Etten E, Branisteanu DD, Overbergh L, Bouillon R, Verstuyf A, Mathieu C. 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].
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].
Williams DG. C3 nephritic factor and mesangiocapillary glomerulonephritis. Pediatr Nephrol. Feb 1997;11(1):96-8. [Medline].

