Resumo

SOUSA, Júnia Bicalho de; CARVALHO, Sílvia Pimenta de; PEREIRA, Luciana Baptista  e  VALE, Everton Siviero do. Do you know this syndrome?. An. Bras. Dermatol. [online]. 2006, vol.81, n.1, pp.87-90. ISSN 1806-4841.  https://doi.org/10.1590/S0365-05962006000100012.

Congenital generalized lipodystrophy (Berardinelli-Seip syndrome) is a recessive autossomic disease characterized by absence of subcutaneous tissue. The fat tissue absence leads to metabolic dysfunction of lipids and carbohydrates, peripheral insulin resistance and increased seric levels of triglycerides and also a higher metabolic rate. Other findings are acanthosis nigricans, acromegaly, hepatomegaly and muscular, bony, cardiovascular and neurological abnormalities. A case of a patient with this syndrome is reported, whose diagnosis was made in a dermatology ambulatory.

Palavras-chave : Acanthosis nigricans; Acromegaly; Hiperinsulinism; Hipertriglyceridemia; Insulin resistance; Lipodystrophy.

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