Acquired Lipodystrophy

Lipodystrophy refers to the loss of adipose tissue. It is classified as either diffuse (generalized) or local (partial) and results from either genetic or acquired etiologies. Ectopic fat in tissues such as liver and muscle may be increased. The cumulative loss of fat leads to a decrease in adipose-derived adiponectin and leptin. Circulating levels of these adipokines are lower in generalized versus partial lipodystrophy as predicted from the loss of fat.

Lipodystrophy is a problem with the way your body uses and stores fat. It’s called acquired when you aren’t born with it. It often affects the fat that’s just under your skin, so it can change the way you look. It also can cause other changes in your body.

Some people with HIV get lipodystrophy (LD-HIV). It may be related to the medicines they take or the disease itself.

Other acquired lipodystrophies are:

Acquired generalized lipodystrophy (AGL), or Lawrence syndrome

Acquired partial lipodystrophy (APL), also called progressive lipodystrophy or Barraquer-Simons syndrome

Localized lipodystrophy

Generalized lipodystrophy happens throughout the body, while the partial form only affects one area of the body.

If you are infected with HIV (human immunodeficiency syndrome) you have a risk of developing lipodystrophy associated with antiretroviral treatment (for example, protease inhibitors or PIs) for HIV infection. Antiretroviral HIV treatment is directed precisely against the virus that causes HIV.

Many individuals with lipodystrophy have low blood levels of leptin, a hormone produced by fat cells that’s involved in how body fat is regulated.

A range of biochemical processes (metabolic) abnormalities that affect the health of individuals with this syndrome can include insulin resistance, diabetes (diabetes mellitus), and elevated levels of blood lipids (such as high cholesterol).

The drug metreleptin (Myalept) has been approved by the US FDA in conjunction with dietary therapy for the treatment of patients with congenital generalized or have the acquired generalized form of the disease who are leptin-deficient.

This is a rare disease characterized by generalized disappearance of fat occurring during

childhood and adolescence. A total of about 80 cases have been reported. Patients have normal

body fat at birth thus distinguishing them from patients with congenital generalized lipodystrophy.

Females are affected approximately 3 times more often than males. AGL could be due to

panniculitis (inflammation in the fat) or autoimmune diseases, however, in several cases no

causative factor could be identified.

The onset of AGL may also occur following infections such as varicella, measles, pertussis,diphtheria, pneumonia, osteomyelitis, parotitis, infectious mononucleosis, and hepatitis. Usually, the onset of lipodystrophy is gradual over months to years. Eventually generalized and near complete loss of fat may occur in some patients resulting in muscular appearance and prominent superficial veins (veins under the skin). There is loss of fat from the palms and soles also, while retroorbital and bone marrow fat are preserved.

The degree of loss of intraabdominal fat varies. Affected children may have a voracious appetite. Dark velvety pigmentation (acanthosis nigricans) may occur in the xilla and neck, umbilicus, nipples and occasionally hands and feet. Increased linear growth may be seen in the children. Excess body hair, enlargement of genitalia (clitoromegaly) and occasional ovarian cysts (enlarged ovaries) may be seen in the females. Enlargement of liver and spleen is frequently seen. Cirrhosis has been reported in about one fifth of the patients as a late sequela of hepatic steatosis or autoimmune hepatitis.

The patients commonly have moderately severe elevation of blood lipids. Diabetes occurs usually after the onset of lipodystrophy. Diabetes is often difficult to control despite requiring large doses of injectable insulin. Most patients have low serum levels of leptin and adiponectin. Patients with AGL may also develop other autoimmune disorders like vitiligo (light-colored spots on skin), sicca syndrome, rheumatoid arthritis, dermatomyositis, thyroiditis and chronic active hepatitis. The precise mechanism involved in loss of body fat in this disorder however remains unknown but it is likely that autoimmune mechanisms (body defense mechanisms acting against body fat) are involved. Recently, it has been suggested that fat loss occurs due to adipocyte apoptosis mediated by CD95 receptor activation.

Types of AGL:

There are 3 types of AGL:

Panniculitis variety (type 1): The patient presents with painful and inflamed subcutaneous nodules or maculopapular lesions. Upon healing, depressed scars remain but the overlying skin is normal. New nodules appear and there is progression of subcutaneous fat loss.

Autoimmune disease variety (type 2): In this variety, the patients have past or present evidence of autoimmune diseases.

Idiopathic variety (type 3): This is the most common variety. The etiological factor of this variety is not known.

Causes

Often, doctors don’t know what causes acquired lipodystrophy, but some triggers are:

An infection, such as measles, pneumonia, infectious mononucleosis, or hepatitis

A disease where your immune system attacks your body (called an autoimmune disease)

Repeated injections in or pressure on the same place on your body

An injury

For example, people with diabetes who have to inject insulin daily tend to get localized lipodystrophy where they keep giving themselves shots. That’s a good reason to change your injection site often.

APL is believed to be caused because the immune system mistakenly brings about the destruction of fat cells (autoimmune-mediated destruction of adipocytes). More than 80% of affected individuals have low levels in their blood of complement 3, a protein factor that normally plays a role in the body’s immune system response. Affected individuals also have a circulating autoantibody called complement 3-nephritic factor. An autoantibody is an immune protein that mistakenly targets and damages healthy tissue.

Both AGL and APL may be associated with complement proteins, which are specialized proteins found in the blood that help fight off infection and disease. These proteins are also believed to be involved in the metabolic functions associated with body fat (adipose tissue). In affected individuals, these proteins may render fat cells susceptible to improper destruction by the immune system.

The exact reason why therapy with protease inhibitors and reverse transcriptase inhibitors (nucleoside analogues) in individuals with HIV causes lipodystrophy is not fully understood.

Localized lipodystrophy may be caused by the injection of various drugs, such as insulin, into the subcutaneous tissue. Panniculitis, pressure on a specific area of the body, and other mechanisms may also cause localized lipodystrophy.

The underlying issue in individuals with acquired lipodystrophy is the complete or partial loss of adipose tissue. The primary role of adipose tissue is to store fat for energy. Adipose tissue also secretes a variety of molecules that are involved with or influence various hormonal functions. For example, patients with AGL may have reduced levels of leptin, a hormone or cytokine produced by adipose cells which plays a role in controlling appetite by working centrally in the brain and hypothalamus.

Adipose tissue is made up of fat cells (adipocytes). Each adipocyte has a lipid droplet that accounts for approximately 90% of its cell volume. An adipocyte stores fats (triglycerides) within its lipid droplet. Damage to adipose tissue in acquired lipodystrophy prevents proper fat storage. Consequently, fat is lost from adipose tissue and, in some cases, is improperly stored in other tissue of the body such as the liver and skeletal muscle causing symptoms such as liver disease and insulin resistance.

Symptoms

While all kinds of acquired lipodystrophy cause a loss of body fat, exactly what that means for each person is different.The type your child has will affect:

Where it happens

How much fat is lost

Other effects on her body

AGL. She’ll lose fat from under the skin all over her body, including her face, arms, legs, palms, and sometimes the soles of her feet. She could look very muscular, and you’ll be able to see veins under her skin.

Kids with AGL are very hungry all the time and grow fast. Adults may have large hands and feet and a strong, square jawbone if their hormone balance is off and they’ve kept growing. They could have larger than usual sex organs (clitoris and ovaries, penis and testicles).

A woman might have irregular periods or none at all. She could have polycystic ovary syndrome (PCOS). She’ll probably have extra hair on her upper lip and chin.

Some people with AGL have patches of dark, velvety skin in their underarms, on their neck, around their belly button or nipples, or on their hands and feet.

Because of the fat loss, a person may have problems using sugar in her body or controlling her blood sugar and triglyceride levels. She could have a large liver or spleen.

APL. This type affects just the upper body, on both sides. It usually starts with her face and moves to her neck, arms, and chest.

Localized. This looks like a dent in the skin, but the skin itself seems fine. The size can vary. It could be in one spot or many. There might also be tender or painful bumps.

Getting a Diagnosis

When you go to the doctor, he’ll do a full exam and ask questions about your child’s health:

What symptoms have you noticed?

When did you first see them?

Are changes in how she looks only in certain areas, or all over?

Has she had panniculitis — swollen bumps under her skin or a bumpy red rash?

Does she have diabetes?

What other health problems or recent injuries has she had?

Have you had her blood sugar, cholesterol, and triglyceride levels checked?

What is acquired lipodystrophy?

Acquired lipodystrophy is a form of the syndrome that is not genetic, but develops as a response to another condition. The acquired forms include:

Acquired generalized lipodystrophy (AGL), or Lawrence syndrome

Acquired partial lipodystrophy (Barraquer-Simons syndrome) is a disorder that leads to loss of fat in the upper trunk and face, which begins in childhood or adolescence.

Lipodystrophy induced by protease inhibitors used in HIV infection treatment; this can involve both fat loss and abnormal fat buildup for the patient with HIV.

Localized, or partial, lipodystrophy, such as at an injection site (for example, from cortisone injection), which typically does not affect the overall health of the patient.

Treatment

Since you can’t replace the missing body fat, your aim will be to avoid complications of the disease. A healthy lifestyle plays a big role.

Everyone with lipodystrophy should eat a low-fat diet. But children still need enough calories and good nutrition so they grow properly. Exercise will help your child stay healthy, too. Physical activity lowers blood sugar and can keep fat from building up dangerously.

People with AGL may be able to get metreleptin injections (Myalept) to replace the missing leptin and help prevent other diseases. Statins and omega-3 fatty acids, found in some fish, can help control high cholesterol or triglycerides, too.

If your child has or gets diabetes, she’ll need to take insulin or other drugs to control her blood sugar.

Women with AGL shouldn’t use oral birth control or hormone replacement therapy for menopause because they can make levels of certain fats worse.

Taking Care of Your Child

Because this condition affects how you look, care and compassion are as important as medicine. Focus on keeping your child healthy and being supportive.

Set the tone for others. Be positive and open-minded. People may not know how to react or what to say to keep from prying, or offending or embarrassing you and your child. When someone asks about her, be matter-of-fact about her condition.

Do what you can to boost her self-esteem. Try to focus your praise on achievements, rather than appearance.

Encourage friendships. But kids will be kids, so prepare her for unkind looks and words. You can help her practice how she’ll react with role-play and humor.

Consider professional counseling. Someone with training can help your child and your family sort out their feelings as they deal with the challenges of this disease.

What to Expect

Over time, people with AGL can lose most or all of their body fat. With APL, it typically stops after a few years.

In general, the more fat you lose, the more serious the condition is. Yet many people with lipodystrophy live active, productive lives.

You’ll need to work closely with your doctor to prevent complications. For example, people with AGL are likely to have heart and liver problems. Diabetes can be hard to control. They might develop autoimmune disorders such as vitiligo (light-colored spots of skin), rheumatoid arthritis, and a kind of hepatitis.

Generalized lipodystrophy

In generalized lipodystrophy, fat can be found in abnormal locations in the body including the heart, kidneys, liver, and pancreas. The presence of fat deposits in these organs can cause problems such as:

Insulin resistance

Diabetes

High cholesterol

Fatty liver disease

Pancreatitis

Heart disease

What is the prognosis and lifespan for a person with lipodystrophy?

It is difficult to estimate the health prognosis for a given person with lipodystrophy because there are many different kinds of the disorder. Moreover, the health of people is affected to varying degrees. Prognosis or outcome on the patient health will be dependent upon the severity of the disorder as well as the extent of associated problems, such as diabetes, HIV, or other diseases and health problems.

Prognosis is also dependent upon the treatment of any associated health conditions. to estimate the health prognosis for a given person with lipodystrophy because there are many different kinds of the disorder. Moreover, the health of people is affected to varying degrees. Prognosis or outcome on the patient’s health will be dependent upon the severity of the disorder as well as the extent of associated problems, such as diabetes, HIV, or other diseases and health problems. Prognosis is also dependent upon the treatment of any associated health conditions.

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