Table 1.

Classification, clinical features, and etiology of the four major subtypes of lipodystrophy

	Generalized		Partial
	Genetic	Acquired	Genetic	Acquired
Subtype	Congenital generalized lipodystrophy	Acquired generalized lipodystrophy	Familial partial lipodystrophy	Acquired partial lipodystrophy
Genes for autosomal recessive subtypes	AGPAT2, BSCL2, CAV1, CAVIN1 [10–13]	None	CIDEC, LIPE, PCYT1A [14–18]	None
Genes for autosomal dominant subtypes	None	None	LMNA, PPARG, AKT2, PLIN1, ADRA2A [19–24]	None
Pathogenesis	Inability to store triglycerides in adipose tissue	Autoimmune, panniculitis, idiopathic (perilipin 1 autoantibody)	Various different mechanisms	Autoimmune (Complement 3 nephritic factor autoantibody)
Lipodystrophy phenotype	Near total absence of body fat since birth or soon after	Gradual loss of subcutaneous fat from nearly entire body	Absence of fat from the limbs	Absence of fat from the upper body, including the face, neck, arms and chest
Metabolic complications	+	+	+	Mild or none

⁺Present