Lipodystrophy

Overview

Lipodystrophy or lipoatrophy is primary idiopathic atrophy of adipose tissue. Lipodystrophy is a very rare disorder of unknown etiology.

Symptoms

Lipodystrophy can be total, partial, or localized. Total lipodystrophy consists of congenital or acquired complete loss of adipose tissue usually associated with hepatomegaly, hyperglycemia, insulin resistance, hyperlipidemia, and hypermetabolism.

Partial lipodystrophy is manifested as symmetric loss of facial fat tissue with or without similar atrophy of the arms and upper part of the trunk. This syndrome has been associated with renal disease, glomerulonephritis, diabetes, hirsutism, hyperlipidemia, hypocomplementemia, and immunological disorders.

Localized lipodystrophy is localized loss of adipose tissue usually involving multiple areas. Lesions are multifocal, well-demarcated, and atrophic. Lesions can be pigmented or nonpigmented, and they usually are painless. Lesions can be as big as 15 cm in diameter. Lesions tend to be located proximally, ie, buttocks, thighs, and upper arms are involved more frequently than lower legs are. The face, scalp, breasts, distal aspects of the extremities, and mucous membranes tend to be spared. Some patients have local panatrophy involving muscle, fat, and morphealike changes. Local panatrophy can present as panatrophy that includes the manifestation of hemifacial atrophy. One subset group has annular atrophy of the ankles and semicircular lipoatrophy of the anterolateral region of the thighs.

Causes

The cause of lipodystrophic syndromes is unknown. One subset of the lipodystrophic syndromes is associated with subcutaneous and intradermal injection sites. In this group, trauma may induce the release of macrophage cytokines (eg, tumour necrosis factor, interleukin-1) that might enhance lipocyte catabolism. Impure animal insulin might lead to localized lipodystrophy, possibly because of a cross-reaction with lipid tissues and insulin antibody. Localized lipodystrophy caused by a cross-reaction is very rare with synthetic insulin.

Lipodystrophy is quite often associated with HIV, which typically refers to changes in fat distribution that are often associated with metabolic abnormalities, including dyslipidemia and insulin resistance. Specifically, patients with lipoatrophy have loss of subcutaneous fat, most noticeably in the limbs, face, and/or buttocks areas. Patients with fat accumulation have gain of visceral fat in the abdomen and may have dorsocervical fat pad enlargement (buffalo hump) and breast enlargement.

 

Source: Medscape

Diagnosis

In addition to physical exam of your fat tissue (Skinfold-thickness test), the following tests can be performed to confirm Lipodystrophy:

  • Metabolic tests (triglyceride and glucose levels in blood)
  • Liver function tests (AST, ALT, ultrasound)
  • Kidney function tests (serum creatinine levels in blood)
  • Cardiovascular (Electrocardiography, Echocardiography, Stress test).

 

Source: My Lipodystrophy

 

Treatment

In general, treatment for acquired partial lipodystrophy is limited to cosmetic, dietary, or medical options.

  • Surgery (fat transplant, facial reconstruction, liposuction) is mainly done for cosmetic purposes.
  • Diet: No evidence in the literature favors any specific diets in this group of patients. A low-fat, high-carbohydrate diet can be detrimental with regard to triglyceride levels, and weight gain should be avoided to reduce the risk of worsening metabolic status. However, children with this syndrome should be permitted normal food intake to allow for normal growth.
  • Few drugs (thiazolidinediones and its derivatives) were used for management of Lipodystrophy, however patients should be informed about side effects.

Currently, no effective treatment exists to halt the progression of lipodystrophy.

 

Source: Medscape

 

Resources

My Lipodystrophy