Acquired generalized lipodystrophy (AGL), also known as Lawrence syndrome[1] and Lawrence–Seip syndrome,[1] is a rare skin condition that appears during childhood or adolescence, characterized by fat loss affecting large areas of the body, particularly the face, arms, and legs.
[2]: 496 There are four types of lipodystrophy based on its onset and areas affected: acquired or inherited (congenital or familial), and generalized or partial.
Both acquired or inherited lipodystrophy present as loss of adipose tissues, in the absence of nutritional deprivation.
[7] As subcutaneous fat is lost, affected areas show prominent structures of veins and muscle.
[6][11] Panniculitis is characterized by inflammatory nodules of the subcutaneous fat, and in this type of AGL, adipose destruction originates locally at the infection or inflammation site and develops into generalized lipodystrophy.
Continuous elevation in triglyceride levels further contributes to metabolic problems including insulin resistance.
[13] Diagnosis is made comprehensively, together with visual observation, body fat assessment, a review of lab panels consisting of A1c, glucose, lipid, and patient history.
Caliper measurements of skinfold thickness is recommended to quantify fat loss as a supportive information.
[7] Less commonly, biphotonic absorptiometry and magnetic resonance imaging (MRI) can be done for the measurement of body fat.
Specifically, the diagnosis is strongly considered for those requiring ≥200 units/day of insulin and persistent elevation of ≥250 mg/dl of triglyceride levels.
[12] Anti-diabetic medications such as insulin, metformin, or thiazolidinediones are used for insulin-resistance or high glucose levels, or statins or fibrates are used for hyperlipidemia.
[12] Plasmapheresis was previously an option for lowering extremely high triglyceride levels for preventing pancreatitis and painful xanthoma, but its use has been decreased after the approval of metreleptin.
[5] Much research for the treatment of lipodystrophy focuses on the safety and efficacy of leptin replacement therapy and the outlook is positive in many studies.
[citation needed] According to a prospective, open-label clinical study at the NIH, metreleptin decreased the fasting glucose level from 180 mg/dL to 121 mg/dL, HbA1c from 8.4% to 6.4%, total cholesterol from 214 mg/dL to 146 mg/dL, and triglycerides from 467 (200-847)mg/dL to 180 (106-312)mg/dL after 12 months of use (p<0.001).
Patients reported improved quality of life and reduced need for other medications without significant adverse effects.