A Case Report Of Acquired Partial Lipodystrophy And Literature Review

Background and ObjectivesLipodystrophy, which is called lipodystrophic syndrome, is an umbrella term to describe a group of infrequent diseases characterized by lossing of adipose tissue combing with metabolic disorders. It can be divided into four types by the etiology and the pattern of lossing, which are congenital generalized lipodystrophy, acquired generalized lipodystrophy, familial partial lipodystrophy and acquired partial lipodystrophy. Acquired partial lipodystrophy is a progressing disease with the sequence that adipose lossing from cephalosome to distal part. Subcutaneous fat of legs and abdomen are normal or accumulated in some times. Metabolic abnormalities, such as hyperinsulinemia, diabetes and hypertriglyceridemia are observed in patients with lipodystrophy. The purpose of the study is to diagnose a case of acquired partial lipodystrophy by physical examination, laboratory detection and determination of a gene, observe the treatment effect by following up physical symptoms and metabolic index after oral drugs and improve the recognizations in diagnosis and therapy of acquired partial lipodystrophy.Subjects and MethodsA 17-year-old man with facial emaciation, increasing wrinkles and chromatodermatosis.1. After a general physical examination, we find the patient have a generally muscular appearance and peripheral lipodystrophy; Acanthosis nigricans are preserved in the nuchal, axillary and inguinal and folds; Excess adipose tissue are accumalated in abdomen and lower limbs; His reproductive organ developed normally.2. Hematologic examination:blood routine, liver and kidney function, biochemistry examination; oral glucose tolerance test, the rhythm of cortisol, the function of thyroid, C peptide and insulin releasing test, sex hormones; immunal test, virus (such as hepatitis and HIV); Detecting the four genes(LMNA, PPARG, AKT2, CAV1) to except familial partial lipodystrophy.3. Imaging examination:whole body MRI scanning, Body Composition Analysis, X-ray of left wrist, ultrasound of cardiac and abdominal. The aim is to understand the distribution of adipose and complications.4. Educating the patient to have a diet and treating complications by oral drugs, such as metformin, pioglitazone, losartan potassium, metoprolol tartrate tablets, nephritis rehabilitation pieces.5. Following up:examing the metabolic index:blood glucose, insulin, c-peptide, hepatic function, uric acid, urine protein and adjusting the doses of the drugs.6. Considering literature to discuss the diagnosis, differential diagnosis, pathogenesis and treatment of the disease.ResultsAccording to clinical manifestation and examination, we have the following diagnoses:1. Acquired Partial Lipodystrophy, Insulin Resistance Syndrome, Acanthosis Nigricans, Impaired Glucose Tolerance, Dyslipidemia, Fatty liver disease (Nonalcoholic Fatty Liver Disease) 2. High Blood Pressure (grade 1, low-risk) 3. Obstructive Sleep Apnea-hypopnea Syndrome(OSAHS). After 2 months follow-up, patients with weight lossing 2 kg. acanthosis nigricans is still visible, the colour of the skin becomes lighter, insulin resistance improved, urine protein reduced. After 5 months, the weight lossed 3 kg. the colour of the skin and laboratory results haven’t changed. After 8 months, the weight lossed 5kg, the patient haven’t appeared adverse reaction.Conclusion1. We should consider acquired partial lipodystrophy and detect the genes if patients with facial emaciation, lossing of adipose in the upper extremities and accumulating excess adipose in the lower extremities, severe insulin resistance.2. Metformin and pioglitazone are useful in improving insulin resistance and regulating metabolism in short time. However, the effect in lipodystrophy is insignificant. A further research is vital to observe the long-period effect.