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Benign symmetric lipomatosis
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Benign symmetric lipomatosis

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Contributors: Jennifer Yeh PhD, Susan Burgin MD
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Synopsis

Benign symmetrical lipomatosis (BSL; multiple names including multiple symmetric lipomatosis, Madelung disease, Launois-Bensaude syndrome, symmetrical adenolipomatosis) is a rare disorder (incidence approximately 1 in 25 000) characterized by multiple symmetric and disfiguring abnormal fat depositions on the neck, shoulders, upper extremities, and upper trunk.

BSL is more prevalent in males (male to female ratio as high as 15:1) from Mediterranean or eastern European countries (Italy, Spain, France). Patients typically present between age 20 and 40.

It is thought that BSL may result from functionally defective brown adipose tissue. While chronic alcohol ingestion is the most commonly encountered association, there are reports in families with an autosomal dominant pattern and pediatric cases have been reported. BSL has also been associated with metabolic disease (impaired fasting glucose, diabetes, and hyperlipidemia) in obese patients.

Three types of lipoma distribution may occur.

Type 1: "Madelung collar" or "horse collar"
  • More common in men
  • Fat accumulation on neck, submental region, upper back
  • Difficulty turning head
  • Deep infiltration of mediastinal and pharyngeal structures leading to dyspnea, obstructive sleep apnea, dysphagia, dysphonia, superior vena cava compression.
Type 2: Pseudoathletic type
  • More common in women
  • Diffuse fat accumulation on trunk and proximal extremities
Type 3: Gynecoid type
  • Large bilateral breast masses
  • Lipomatous deposits have also been reported in the tongue (macroglossia), scrotum, and retro-orbital space.
BSL has an initial period of fast growth followed by slow progression or stability. Fat deposits increase in size over years, many reaching significant size to become cosmetically disfiguring or to cause dyspnea, dysphagia, dysphonia, or superior vena cava syndrome depending on the location of fatty deposits. BSL is also associated with sensory, motor, and autonomic polyneuropathy (85% of patients).

For more information, see OMIM.

Codes

ICD10CM:
E88.89 – Other specified metabolic disorders

SNOMEDCT:
254832003 – Benign symmetric lipomatosis

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Differential diagnosis for neck masses:
Pitfalls:
  • Large lipomas can mimic head and neck malignancy.
  • BSL is sometimes mistaken for truncal obesity or Cushing syndrome.

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Therapy

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References

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Last Updated: 12/18/2018
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Benign symmetric lipomatosis
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Benign symmetric lipomatosis (Madelung Collar) : Alcohol use, Neck, Upper back, Neck mass, Dysphagia, Dysphonia, Dyspnea, Shoulders
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