Definition

What is Barraquer-Simons syndrome?

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.

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.

How common is Barraquer-Simons syndrome?

Barraquer-Simons syndrome is not common. It affects females more often than males. Please discuss with your doctor for further information.

Symptoms

What are the symptoms of Barraquer-Simons syndrome?

The common symptoms of Barraquer-Simons syndrome are:

  • Lipoatrophy
  • Autoimmunity
  • Decreased serum complement C3
  • Hearing impairment
  • Intellectual disability
  • Lymphocytosis
  • Myopathy
  • Progeroid facial appearance
  • Seizures
  • Arthralgia
  • Generalized hirsutism
  • Glomerulopathy
  • Hepatic steatosis
  • Immunodeficiency
  • Insulin resistance
  • Microscopic hematuria
  • Proteinuria
  • Abnormality of lipid metabolism
  • Autosomal dominant inheritance
  • Diabetes mellitus
  • Hematuria
  • Hirsutism
  • Juvenile onset
  • Loss of subcutaneous adipose tissue from upper limbs
  • Loss of truncal subcutaneous adipose tissue
  • Membranoproliferative glomerulonephritis
  • Nephrotic syndrome
  • Phenotypic variability
  • Polycystic ovaries
  • Progressive loss of facial adipose tissue
  • Recurrent infections
  • Sporadic

There may be some symptoms not listed above. If you have any concerns about a symptom, please consult your doctor.

When should I see my doctor?

If you have any signs or symptoms listed above or have any questions, please consult with your doctor. Everyone’s body acts differently. It is always best to discuss with your doctor what is best for your situation.

Causes

What causes Barraquer-Simons syndrome?

Please consult with your doctor for further information.

Risk factors

What increases my risk for Barraquer-Simons syndrome?

Onset usually begins in childhood following a viral illness.

Diagnosis & treatment

The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.

How is Barraquer-Simons syndrome diagnosed?

Please consult with your doctor for further information.

How is Barraquer-Simons syndrome 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.

Lifestyle changes & home remedies

What are some lifestyle changes or home remedies that can help me manage Barraquer-Simons syndrome?

If you have any questions, please consult with your doctor to better understand the best solution for you.

Hello Health Group does not provide medical advice, diagnosis or treatment.

Sources

Review Date: January 25, 2018 | Last Modified: January 25, 2018

Want to live your best life?
Get the Hello Doktor Daily newsletter for health tips, wellness updates and more.