Table 2.

When to suspect lipodystrophy

	Generalized lipodystrophy	Partial lipodystrophy
Family history	Positive family history in siblings only (autosomal recessive inheritance)	Positive family history in multiple generations (autosomal dominant inheritance) Positive family history in siblings only (autosomal recessive inheritance)
Clinical features	• Generalized Muscularity with loss of body fat	• Muscular extremities with loss of subcutaneous fat
	• Prominent veins	• Cushingoid habitus
	• Voracious appetite	• Mild features in men
	• Acromegaloid features • Prominence of umbilicus	• Increased fat in the face, neck, dorsocervical, perineal and intra-abdominal regions
	• Hepatomegaly
	• Acanthosis nigricans	•Hepatomegaly
	• Irregular menstrual periods with polycystic ovaries	• Irregular menstrual periods with polycystic ovaries
Metabolic features	• Autoantibody negative diabetes with evidence of severe insulin resistance*: high insulin requirement for metabolic control
	• Severe acanthosis nigricans
	• Severe hypertriglyceridemia (triglycerides ≥ 500 mg/dL) and history of acute pancreatitis associated with hypertriglyceridemia
	• Steatohepatitis and hepatomegaly

^*There have been few case reports of type 1 diabetes in association with AGL