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.
Surgical Care
Various surgical techniques have been adopted through the years in an effort to improve progressive lipodystrophy patients’ facial appearance. [27, 28] 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. [4] It may be useful in selected patients for the reconstruction of bilateral facial atrophy.
Consultations
Referral to a nephrologist or an internist may be warranted for progressive lipodystrophy patients with severe nephropathy and those with associated autoimmune diseases.
Diet
Hyperalimentation can result in the excessive accumulation of fat in an unaffected area with no improvement on dystrophic areas.
Long-Term Monitoring
Note the following:
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Renal status: Patients should be monitored regularly for evidence of glomerulonephritis. Glomerulonephritis can develop more than 10 years after the onset of progressive lipodystrophy.
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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. [29] Other autoimmune disorders have also been associated with this disease.
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Model of the adipocyte destruction in acquired partial lipodystrophy showing complement activation at the adipocyte surface resulting in adipocyte lysis. Adipocytes synthesize C3, factor B, and factor D (adipsin), which allows C3bBb to be formed locally, but which usually does not result in the activation of the terminal lytic part of the complement pathway (C5-9).The IgG antibody, C3Nef, prevents the alternative complement C3-convertase C3Bb from dissociative inactivation, resulting in adipocyte lysis. Adipocytes synthesize factor D, the limiting component of the alternative complement pathway, which cleaves C3-bound factor B to its active enzymatic form. Factor D is expressed to a higher extent in the fat cells of the upper half of the body compared with the lower half, and it is possibly this regional difference that accounts for the restriction of fat loss to the head, arms, and trunk. C3Nef is also associated with type II, dense-deposit membranoproliferative glomerulonephritis in which subendothelial deposits of immunoglobulin and C3 are probably due to a deregulated alternative complement pathway.
