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Barraquer-Simons syndrome in systemic lupus erythematosus: A case report
Suman Acharya1, Suraj Kandel2, Suraj Shrestha2
1Department of Internal Medicine Tribhuvan University Teaching Hospital Kathmandu Nepal.
Clinical Case Reports
|December 1, 2021
Summary
Barraquer-Simons syndrome causes progressive fat loss in the face and upper body. Long-term monitoring is crucial for managing associated autoimmune diseases and potential kidney complications.
Area of Science:
- Rare genetic disorders
- Dermatology
- Nephrology
Background:
- Barraquer-Simons syndrome is a rare genetic disorder.
- Characterized by progressive lipodystrophy affecting the face and upper body.
- Often associated with autoimmune conditions like systemic lupus erythematosus.
Observation:
- Patients experience significant loss of subcutaneous adipose tissue.
- The condition can manifest with various metabolic disturbances.
- Renal complications and other systemic diseases may be present.
Findings:
- Progressive lipodystrophy is the hallmark of Barraquer-Simons syndrome.
- Association with autoimmune diseases necessitates comprehensive patient evaluation.
- Metabolic and renal issues require diligent, long-term management.
Implications:
- Early identification of Barraquer-Simons syndrome is vital for timely intervention.
- Close monitoring can prevent severe metabolic and renal complications.
- Understanding associated conditions improves patient outcomes and quality of life.
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