Lipodystrophy is a disease that causes severe loss of body fat, often leading to insulin resistance, diabetes, fatty liver, and high triglyceride levels. There are genetic and acquired forms, with treatments including cosmetic procedures and medication to manage associated health problems. Prognosis is not ideal, with those with the congenital form typically living to be young adults or middle-aged. Research is ongoing to improve outcomes.
Lipodystrophy is a disease that affects the fatty tissue of the body and is mainly characterized by a severe loss of body fat. People with lipodystrophy often develop insulin resistance and have diabetes, fatty liver, and high triglyceride levels. There are different types of disease; some are genetic and some are acquired.
If a patient has a genetic form of lipodystrophy, they may have a condition called Beradinelli-Seip syndrome. It is a congenital disease that affects one in 12 million people in the United States, one in 1 million people in Norway, one in 500,000 people in Portugal, and one in 200,000 people in Lebanon. In infants, it often occurs when the newborn fails to thrive, or has gigantism, hollow cheeks, an enlarged tongue, or developmental delays. Other genetic forms of the disease include familial partial lipodystrophy Dunnigan type, familial partial lipodystrophy Kobberling type, and mandibulacral type dysplasia.
Alternatively, a patient may suffer from the acquired form of lipodystrophy. The acquired form can be divided into several types. For example, acquired generalized lipodystrophy may include Lawrence syndrome, which is usually preceded by an infection or disease and involves extreme loss of body fat. There is also an acquired partial form of lipodystrophy, such as Barraquer-Simons syndrome, in which the patient suffers from extreme fat loss in the face, torso and arms while suffering from fatty hypertrophy – or excess fat – in the legs. One of the most common forms of the disease is seen in patients who are being treated for HIV with antiretroviral drugs. They often have abnormal accumulation of fat in the torso, while simultaneously experiencing extreme fat loss in other localized areas.
While there is no treatment for lipodystrophy in most cases, the symptoms and related conditions can be treated. Treatments can be cosmetic, such as fat transplants, facial reconstruction and liposuction. Treatments may also include medications to manage diabetes, raised triglyceride levels, or other associated health problems. However, there is no way to stop the progression of the disease. Surgical options, such as those mentioned above, are not recommended for children; however, they may be a good solution for those who experience deformities later in life.
Unfortunately, at this time, the prognosis is not ideal for those with lipodystrophy. People with the congenital form of the disease will typically live to be young adults or middle-aged. Alternatively, if a patient has the acquired form of the disease, she may live a little longer and usually survives into middle age. However, research is currently underway to improve the scenario of those affected by the life-threatening condition.
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