Barraquer-Simons syndrome

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Other Names: APL, Lipodystophy partial progressive, Lipodystrophy cephalothoracic type, Lipodystrophy partial acquired See more

Categories: Congenital and Genetic Diseases, Endocrine Diseases, Skin Diseases

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Description

Barraquer-Simons syndrome , or acquired partial lipodystrophy, is characterized by the loss of fat from the face, neck, shoulders, arms, forearms, chest and abdomen. Occasionally the groin or thighs are also affected. Onset usually begins in childhood following a viral illness. It affects females more often than males. The fat loss usually has a 18 month course, but can come and go over the course of several years. Following puberty, affected women may experience a disproportionate accumulation of fat in the hips and lower limbs. Around 1 in 5 people with this syndrome develop membranoproliferative glomerulonephritis. This kidney condition usually develops more than 10 years after the lipodystrophy's onset. Autoimmune disorders may also occur in association with this syndrome.

Source: GARD Last updated on 05-01-20

Symptoms

Symptoms and their Known Frequencies

We're currently performing maintenance on our symptoms information from GARD. Please check back at a later time for updates.

What is the long term outcome for Barraquer-Simons syndrome?

Barraquer-Simons syndrome is a disease that progresses slowly. It causes a loss of fat tissue in the face, which can affect facial features. About 20 percent of people develop a type of kidney disease called membranoproliferative glomerulonephritis, which can cause serious complications. Rarely, Barraquer-Simons syndrome can cause insulin resistance.

Last updated on 05-01-20

How might Barraquer-Simons syndrome be treated?

Surgery may be used to improve a person's appearance, but is not needed for medical reasons. Facial reconstruction techniques may be used with varying success. These techniques may include transplantation of fat tissue, silicone implants, movement of facial muscles, or other techniques.

No specific diet is recommended for people with Barraquer-Simons syndrome and weight gain should be avoided. Regular exercise is recommended to improve a person's metabolic status.

If a person with Barraquer-Simons syndrome has kidney problems, then they may also need to be managed. Treatment may involving a special diet or medications. Dialysis or a kidney transplant may be needed if the condition progresses to kidney failure.

Last updated on 05-01-20

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