Progressive Lipodystrophy

Progressive (cephalothoracic) lipodystrophy (Barraquer-Simons syndrome) is a rare acquired loss of subcutaneous adipose tissue from the face/neck/upper trunk, often with relative lower-body fat accumulation, low complement (C3), and membranoproliferative glomerulonephritis/nephrotic syndrome. It predominantly affects women. There is no specific therapy; manage renal disease and metabolic complications.

Common Symptoms

Gradual loss of facial/upper-body subcutaneous fat; lower-body fat accumulation; low complement; nephrotic syndrome

Causes and Pathogenesis

Complement-mediated/autoimmune adipocyte lysis causes partial lipoatrophy; associated C3 nephritic factor and renal disease.

Treatment

No specific therapy; manage glomerulonephritis/nephrotic syndrome; cosmetic reconstruction

Preventive Measures

Screen complement/renal function; treat renal disease.

Department to Visit

It is recommended to visit the Endocrinology department; an appointment can be booked on this platform before the visit.

Warm reminder: The above information is for health science reference only and cannot replace professional medical diagnosis. If you have related symptoms, please seek medical attention at a regular hospital promptly.