Lipodystrophy Congenital Generalized Type 5 C5882745
A rare disorder causing near-complete loss of body fat, affecting metabolism and fat storage. It leads to severe insulin resistance and fat buildup in other organs. Treatment includes metabolic management and dietary approaches.
Key facts at a glance
- ICD-10
- E88.9
- ICD-11
- 5A00.0
- Body system
- metabolic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Fatigue or weight changes
(metabolic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Loss of subcutaneous fat (generalized lipodystrophy)
(metabolic)Severe insulin resistance
(metabolic)Hypertriglyceridemia
(metabolic)Hepatomegaly
(metabolic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Leptin Replacement Therapy
Metabolic therapy using metreleptin to address severe leptin deficiency common in generalized lipodystrophy, which helps improve insulin sensitivity and reduce hyperglycemia and hypertriglyceridemia.
Medication management with follow-up
Active treatment with regular monitoring by a healthcare provider is recommended.
Controlled Carbohydrate Diet
Structured nutritional plan emphasizing complex carbohydrates, reduced simple sugars, and adequate protein to manage insulin resistance and dyslipidemia characteristic of the condition.
Additional treatment options
Metabolic Syndrome Surveillance
Comprehensive monitoring of glucose levels, lipid panels, liver function, and cardiovascular markers to detect complications early and guide treatment adjustments.
monitoringCosmetic Fat Redistribution Surgery
Surgical procedures including fat grafting or filler injections to address severe fat loss and improve body contour, though this does not treat underlying metabolic abnormalities.
procedureStructured Exercise Program
Individualised physical activity regimen combining aerobic exercise and resistance training to improve insulin sensitivity, maintain muscle mass, and support metabolic health.
lifestyleWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Lipodystrophy Congenital Generalized Type 5 C5882745?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
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This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 27, 2026. · Read our editorial policy