Generalized lipodystrophy

This code describes a rare, inherited or acquired disorder characterized by a near-total or complete absence of adipose tissue throughout the body. Patients typically present with a distinctive body habitus, severe insulin resistance, hypertriglyceridemia, and hepatic steatosis due to the inability to store fat.

Use this code when documentation clearly indicates a diagnosis of generalized lipodystrophy, encompassing both congenital (Berardinelli-Seip syndrome) and acquired forms (e.g., associated with autoimmune conditions). This diagnosis is often supported by physical examination findings, metabolic abnormalities, and sometimes genetic testing.

• Differentiate from partial lipodystrophy, which involves localized fat loss and has distinct ICD-10-CM codes.
• Always code any associated metabolic complications (e.g., diabetes mellitus, hyperlipidemia, nonalcoholic fatty liver disease) separately and in addition to this code.
• Ensure the documentation specifies "generalized" to avoid confusion with other lipodystrophy types.