Acquired Partial Lipodystrophy Associated With Hypocomplementemia

Ceyhun Dizdarer, MD, Sema Kalkan, MD, Tugrul Ozcan, MD, Demet Tumay, MD, Safiye Aktas, MD

Disclosures

Lab Med. 2005;36(9):546-549. 

In This Article

Abstract and Introduction

  • Acquired partial lipodystrophy is a rare condition with onset in childhood or adolescence. It is characterized by progressive loss of subcutaneous fat of the face, neck, trunk, and upper extremities in a cephalocaudal fashion and usually is coupled with C3 hypocomplementemia.

  • Insulin resistance and its accompanying complications appear to be infrequent. Women are affected approximately 3 times more often than are men.

  • Owing to its rarity in male patients, we are presenting the case of acquired partial lipodystrophy in a 12-year-old boy with a granular accumulation of C3 in the basement membrane on skin biopsy.

The lipodystrophy syndromes are a heterogeneous group of syndromes characterized by selective loss of fat from various parts of the body.[1] They are classified into 2 major types: familial and acquired. The main subtypes of familial lipodystrophies are congenital generalized lipodystrophy, an autosomal recessive disorder characterized by near complete lack of metabolically active adipose tissue from birth, and familial partial lipodystrophy, Dunnigan type, an autosomal dominant disorder characterized by loss of subcutaneous fat from the extremities at puberty and excess fat accumulation in the face and neck. Patients with acquired generalized lipodystrophy have generalized loss of subcutaneous fat, but those with acquired partial lipodystrophy have fat loss limited to the face, trunk, and upper extremities.[2] ( Table 1 )

Acquired partial lipodystrophy is a rare condition with approximately 250 described patients of various ethnic origins.[3] This variety (like acquired generalized lipodystrophy) occurs approximately 3 times more often in women, begins during childhood, and has underlying autoimmunity.[2]

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