Familial partial lipodystrophy type 1

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: Familial partial lipodystrophy, Köbberling type; Köbberling type familial partial lipodystrophy.

Overview

Köbberling type familial partial lipodystrophy is a member of the group of lipodystrophy syndromes characterised by anomalies of adipose tissue distribution and often associated with severe insulin resistance manifested by hyperinsulinemia (insulin levels above 20mUI/l), carbohydrate intolerance or diabetes, hypertriglyceridemia with low levels of high-density lipoproteins (HDL) and arterial hypertension. Acanthosis nigricans, hepatic steatosis and polycystic ovary syndrome are also frequent.

Pathophysiology

Genetics

The mode of transmission is autosomal dominant

Causes

The underlying cause of the disease is unknown.

Epidemiology and Demographics

Köbberling type familial partial lipodystrophy is rare, with only 16 patients reported between 1975 and 2003.

Natural History, Complications and Prognosis

The risk of cardiovascular disease is probably increased but the long-term prognosis is unknown, particularly for cases with early diagnosis and treatment.

Diagnosis

The syndrome is characterised clinically by a loss of subcutaneous adipose tissue in the lower members but accumulation of truncal fat, a normal distribution of facial adipose tissue and, most prominently, arterial hypertension. Diabetes and hypertriglyceridemia, leading to a risk of acute pancreatitis, has been resported in the majority of cases. As the clinical and biological picture is nondistinctive, the syndrome may remain unrecognised, particularly given the absence of genetic confirmation for the disease and the frequency of android obesity in the general population.

Treatment

In principle, treatment of the insulin resistance should follow the same guidelines as those used for other forms of insulin resistance: physical exercise, insulin-sensitising medication (metformin or glitazones), insulin (or preferably insulin analogues), antihypertensives, follow-up and treatment of the hypertriglyceridemia.