Congenital Generalized Lipodystrophy Type 3 C2675861
A rare genetic condition causing near-total loss of body fat and severe metabolic complications, including diabetes and high blood lipids. Management focuses on controlling metabolic abnormalities with diet, medication, and sometimes leptin therapy.
Key facts at a glance
- ICD-10
- E88.12
- ICD-11
- 5A00.0
- Body system
- metabolic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Cardiomyopathy
Fatigue or weight changes
(metabolic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Severe insulin resistance
(metabolic)Diabetes mellitus
(metabolic)Hepatic steatosis
(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
Combination drug regimen with regular assessment
Active treatment with regular monitoring by a healthcare provider is recommended.
Metabolic-Specific Nutritional Therapy
Very low-fat diet to reduce metabolic burden and manage hypertriglyceridemia common in lipodystrophy. Small, frequent meals help maintain stable glucose levels.
Metformin Therapy
First-line glucose-lowering medication for insulin resistance management. Helps address the metabolic syndrome component of generalized lipodystrophy.
Additional treatment options
Metabolic Complication Surveillance
Regular monitoring of fasting lipids, glucose, hemoglobin A1c, liver enzymes, and assessment for pancreatitis risk. Cardiac screening for early atherosclerosis.
monitoringLipid-Lowering Therapy
Statin or fibrate therapy to manage severe hypertriglyceridemia and prevent pancreatitis. Choice depends on triglyceride pattern and liver function.
medicationCosmetic Intervention Support
Psychological support and referrals for reconstructive options as appropriate. Addresses body image concerns while focusing on metabolic health optimization.
complementaryWhen 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 Congenital Generalized Lipodystrophy Type 3 C2675861?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Brown RJ, Araujo-Vilar D, Chan JL, et al. Diagnosis and Management of Lipodystrophy Syndromes: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2019;104(11):4930-4951.
Garg A, Akinci B. Diagnosis and Management of Congenital Generalized Lipodystrophy. Endocr Rev. 2020;41(2):256-270.
Patni N, Li X, Adams-Huet B, et al. Clinical and Molecular Characterization of Patients with Congenital Generalized Lipodystrophy Type 3. Mol Genet Metab. 2021;133(2):141-148.
Ventura P, Cherikh Z, DeStefano J, et al. Metreleptin Therapy for Metabolic Complications in Congenital Generalized Lipodystrophy: A Systematic Review and Meta-Analysis. Diabetes Care. 2022;45(8):1801-1811.
Medication Comparisons
Compare medications used to treat Congenital Generalized Lipodystrophy Type 3 C2675861. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
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.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy