Benign Symmetric Lipomatosis

Benign symmetric lipomatosis (BSL), also known as Launois-Bensaude syndrome, is a rare condition characterized by the growth of multiple, symmetrical, benign fat deposits in the neck, shoulder, and back regions. It is a slowly progressive condition that mainly affects middle-aged and elderly men, although women can also be affected.

BSL is classified as a type of lipomatosis, which refers to the overgrowth of fatty tissue in the body. Lipomatosis can be either benign or malignant, and BSL is a benign form of the condition. The fat deposits in BSL are usually not painful, but they can cause discomfort or disfigurement, and in some cases, they can also interfere with normal movements or activities.

The exact cause of BSL is unknown, but there are several theories that have been proposed. Some researchers believe that it may be due to an inherited genetic predisposition, while others suggest that it may be caused by hormonal imbalances or other environmental factors.

There are several different types of BSL, which can be classified based on the location and size of the fat deposits. The most common types are:

  1. Cervical BSL: This type affects the neck region and is the most common form of BSL. Fat deposits in this area can cause a characteristic “buffalo hump” appearance and can also compress the spinal cord, leading to neurological symptoms.
  2. Thoracic BSL: This type affects the upper back and chest region and can cause a “hunchback” appearance. In severe cases, it can also interfere with breathing.
  3. Scapular BSL: This type affects the shoulder blades and can cause discomfort or pain in the shoulder region.
  4. Limb BSL: This type affects the arms or legs and is the least common form of BSL.

Causes

The exact cause of BSL is unknown, but several factors have been implicated in its development.

  1. Genetics: Studies have shown that BSL can be inherited in an autosomal dominant manner, suggesting a genetic component in its development.
  2. Hormonal Imbalances: Abnormalities in hormonal regulation, particularly of insulin, have been suggested as a contributing factor to BSL. Insulin resistance and high levels of insulin in the blood can lead to an increase in fat storage.
  3. Obesity: Excessive weight and obesity have been linked to the development of BSL. This may be due to the increased fat storage that occurs in obese individuals.
  4. Metabolic Disorders: Metabolic disorders, such as type 2 diabetes, can lead to an increase in fat storage and contribute to the development of BSL.
  5. Medications: Certain medications, such as corticosteroids and antipsychotics, have been linked to the development of BSL due to their ability to increase fat storage.
  6. Alcoholism: Alcoholism can lead to the development of BSL due to the toxic effects of alcohol on the liver, which can disrupt hormonal regulation and increase fat storage.
  7. Endocrine Disorders: Endocrine disorders, such as hypothyroidism and hypercortisolism, have been linked to the development of BSL due to their effects on hormone regulation and fat storage.
  8. Nutrition: A diet high in fat and sugar can contribute to the development of BSL by increasing fat storage.
  9. Age: BSL is more common in older individuals, and the risk of developing the condition increases with age.
  10. Gender: BSL is more commonly diagnosed in men than in women, although the exact reason for this gender disparity is unknown.
  11. Ethnicity: BSL is more commonly diagnosed in individuals of Mediterranean, Middle Eastern, and South Asian descent.
  12. Family History: A family history of BSL can increase the risk of developing the condition.
  13. Smoking: Smoking has been linked to the development of BSL due to its effects on hormone regulation and fat storage.
  14. Stress: Chronic stress can disrupt hormone regulation and increase fat storage, contributing to the development of BSL.
  15. Sleep Disorders: Sleep disorders, such as sleep apnea, have been linked to the development of BSL due to their effects on hormone regulation and fat storage.
  16. Physical Inactivity: A sedentary lifestyle can contribute to the development of BSL by increasing fat storage and disrupting hormone regulation.
  17. Chronic Inflammation: Chronic inflammation has been linked to the development of BSL due to its effects on hormone regulation and fat storage.
  18. Environmental Toxins: Exposure to environmental toxins, such as lead and polychlorinated biphenyls (PCBs), has been linked to the development of BSL due to their effects on hormone regulation and fat storage.
  19. Vitamin Deficiencies: Vitamin deficiencies, particularly of vitamins B6 and B12, have been linked to the development of BSL due to their effects on hormone regulation and fat storage.
  20. Infections: Certain infections, such as human immunodeficiency virus (HIV) and hepatitis C, have been linked to the development of BSL due to their effects on hormone regulation and fat storage.

Symptoms

Symptoms that may be associated with BSL:

  1. Fat deposits: The most obvious symptom of BSL is the presence of multiple, soft, rubbery, benign tumors of fat (lipomas) in various parts of the body, including the neck, back, arms, legs, and abdomen.
  2. Symmetrical distribution: The lipomas tend to develop symmetrically on both sides of the body, which is a characteristic feature of BSL.
  3. Increasing size: The lipomas tend to increase in size over time, and can eventually become quite large, causing physical and cosmetic problems.
  4. Pain: Some people with BSL experience discomfort and pain due to the size and distribution of the lipomas.
  5. Reduced mobility: In severe cases, the size of the lipomas can make it difficult to move and perform everyday activities, such as dressing and grooming.
  6. Weakness: Some people with BSL experience weakness in the affected areas, which can make it difficult to perform physical tasks.
  7. Numbness and tingling: Some people with BSL may experience numbness and tingling in the affected areas, which can be caused by pressure on the nerves.
  8. Difficulty breathing: In severe cases, the size of the lipomas can make it difficult to breathe, especially when lying down.
  9. Heart palpitations: Some people with BSL may experience heart palpitations due to the pressure of the lipomas on the heart and chest.
  10. Digestive problems: Some people with BSL may experience digestive problems, such as bloating, constipation, and abdominal pain, due to the pressure of the lipomas on the abdominal organs.
  11. Fatigue: Some people with BSL may experience fatigue and weakness, which can be related to the physical and emotional strain of living with the condition.
  12. Depression: Some people with BSL may experience depression and anxiety due to the physical and cosmetic impact of the condition.
  13. Insecurity: Some people with BSL may feel self-conscious and insecure about their appearance, which can affect their self-esteem and quality of life.
  14. Difficulty sleeping: Some people with BSL may have difficulty sleeping due to the discomfort and pain caused by the lipomas, as well as the difficulty breathing and pressure on the chest.
  15. Skin irritation: The skin over the lipomas can become irritated and red due to friction and pressure, which can cause discomfort and pain.
  16. Scarring: In some cases, the lipomas can become so large that they cause scarring or disfigurement of the affected areas.
  17. Difficulty performing everyday tasks: In severe cases, the size and distribution of the lipomas can make it difficult to perform everyday tasks, such as dressing and grooming.
  18. Difficulty with physical activity: Some people with BSL may have difficulty participating in physical activities, such as sports or exercise, due to the size and distribution of the lipomas.
  19. Difficulty finding clothing that fits: Some people with BSL may have difficulty finding clothing that fits properly, especially in areas where the lipomas are located.

Diagnosis

Diagnostic tests and procedures used for the diagnosis of BSL:

  1. Physical examination: A physical examination is the first step in diagnosing BSL. The doctor will look for the presence of multiple fat deposits on the neck, trunk, and extremities.
  2. Medical history: The doctor will ask about the patient’s medical history and any family history of BSL or similar conditions.
  3. Ultrasound: An ultrasound uses high-frequency sound waves to create images of the internal organs and soft tissues. It can help to identify the location and size of the fat deposits and determine if they are benign or malignant.
  4. CT scan: A CT scan uses X-rays and computer technology to produce detailed images of the body’s internal structures. It can help to determine the size and location of the fat deposits and any other related conditions.
  5. MRI: An MRI uses magnetic fields and radio waves to produce images of the internal structures of the body. It can provide detailed images of the fat deposits and help to rule out any other underlying conditions.
  6. Biopsy: A biopsy involves removing a small sample of tissue from the fat deposit for examination under a microscope. This test can confirm the diagnosis of BSL and rule out any other underlying conditions.
  7. Complete blood count (CBC): A CBC measures the number of red and white blood cells and platelets in the blood. This test can help to determine if there are any underlying conditions that could be contributing to the fat deposits.
  8. Lipid panel: A lipid panel measures the levels of fats, such as cholesterol and triglycerides, in the blood. This test can help to determine if there are any underlying lipid disorders that could be contributing to the fat deposits.
  9. Hormonal tests: Hormonal tests, such as testosterone and estrogen levels, can be performed to determine if there are any hormonal imbalances that could be contributing to the fat deposits.
  10. Thyroid function tests: Thyroid function tests can be performed to determine if there are any thyroid problems that could be contributing to the fat deposits.
  11. Liver function tests: Liver function tests can be performed to determine if there are any liver problems that could be contributing to the fat deposits.
  12. Renal function tests: Renal function tests can be performed to determine if there are any kidney problems that could be contributing to the fat deposits.
  13. Glucose tolerance test: A glucose tolerance test measures the body’s ability to process glucose and can help to determine if there are any underlying glucose intolerance or diabetes issues.
  14. C-reactive protein test: A C-reactive protein test measures the level of inflammation in the body and can help to determine if there are any underlying inflammatory conditions.
  15. Fasting insulin level: A fasting insulin level measures the amount of insulin in the blood after fasting for at least 8 hours. This test can help to determine if there are any underlying insulin resistance issues.
  16. Adiponectin level: Adiponectin is a hormone produced by adipose (fat) tissue. A test to measure the adiponectin level can help to determine if there are any underlying issues with the fat

Treatment

Treatment options for BSL:

  1. Observation: For mild cases of BSL, observation may be the best course of action. In some cases, the condition may stabilize or even improve on its own over time.
  2. Weight management: Maintaining a healthy weight can help reduce the size of fat deposits and slow down the progression of BSL.
  3. Exercise: Regular physical activity can help improve overall health and burn excess fat.
  4. Liposuction: Liposuction is a surgical procedure that removes excess fat from the body using a suction device. This procedure can be used to treat BSL, but it is not a cure and the fat deposits may return over time.
  5. Fat reduction injections: Injections of drugs that reduce fat, such as deoxycholic acid (Kybella), can be used to treat BSL. However, this treatment is still in the experimental stage and its long-term effects are not yet known.
  6. Lipolysis: Lipolysis is a minimally invasive procedure that uses ultrasound or laser energy to break down fat cells. This procedure can be used to treat BSL, but it is not a cure and the fat deposits may return over time.
  7. Mesotherapy: Mesotherapy is a procedure that involves injecting a mixture of drugs and nutrients into the affected areas to break down fat and improve circulation. This procedure can be used to treat BSL, but it is not a cure and the fat deposits may return over time.
  8. Radiofrequency therapy: Radiofrequency therapy uses high-frequency electromagnetic waves to heat and destroy fat cells. This procedure can be used to treat BSL, but it is not a cure and the fat deposits may return over time.
  9. Cryolipolysis: Cryolipolysis is a non-invasive procedure that uses extreme cold temperatures to destroy fat cells. This procedure can be used to treat BSL, but it is not a cure and the fat deposits may return over time.
  10. Laser-assisted liposuction: Laser-assisted liposuction is a surgical procedure that uses laser energy to melt and remove fat. This procedure can be used to treat BSL, but it is not a cure and the fat deposits may return over time.
  11. Endoscopic thoracic sympathectomy (ETS): ETS is a surgical procedure that involves cutting or destroying the sympathetic nerves that control sweating and other functions. This procedure can be used to treat BSL, but it is not a cure and the fat deposits may return over time.
  12. Liposarcoma excision: Liposarcoma excision is a surgical procedure that involves removing a liposarcoma, a type of malignant tumor that can resemble BSL. This procedure is only necessary if a liposarcoma is suspected.
  13. Steroid injections: Steroid injections can be used to reduce inflammation and swelling in the affected areas.
  14. Anti-inflammatory medications: Nonsteroidal anti-inflammatory drugs (NSAIDs) can be used to reduce pain and inflammation in the affected areas.
  15. Pain management: Pain management techniques
References