Generalized lipodystrophy

Generalized lipodystrophy is a rare, heterogeneous group of metabolic disorders characterized by a widespread or near-total absence of adipose tissue It encompasses both congenital forms, typically presenting in infancy or early childhood due to pathogenic genetic variants, and acquired forms that develop later in life, often associated with autoimmune conditions or panniculitis This ectopic deposition triggers pronounced metabolic derangements across the endocrine and hepatic systems, clinicall...

Generalized lipodystrophy is a rare, heterogeneous group of metabolic disorders characterized by a widespread or near-total absence of adipose tissue Pathophysiologically, the profound deficit of functional adipocytes leads to severe hypoleptinemia and ectopic lipid accumulation throughout non-adipose tissues This ectopic deposition triggers pronounced metabolic derangements across the endocrine and hepatic systems, clinically manifesting as extreme insulin resistance, early-onset diabetes melli...

It encompasses both congenital forms, typically presenting in infancy or early childhood due to pathogenic genetic variants, and acquired forms that develop later in life, often associated with autoimmune conditions or panniculitis Pathophysiologically, the profound deficit of functional adipocytes leads to severe hypoleptinemia and ectopic lipid accumulation throughout non-adipose tissues

Pathophysiologically, the profound deficit of functional adipocytes leads to severe hypoleptinemia and ectopic lipid accumulation throughout non-adipose tissues