Doença de Urbach-Wiethe/proteinose lipoidica.

Abstract

The authors present a case of Urbach-Wiethe's disease (lipoid proteinosis), a rare autosomal recessive disorder, in a 49-year-old female patient with pathognomonic cranial radiological findings demonstrated by radiology and computed tomography. The rarity of this pathology made radiological diagnosis difficult.The authors present a case of Urbach-Wiethe's disease (lipoid proteinosis), a rare autosomal recessive disorder, in a 49-year-old female patient with pathognomonic cranial radiological findings demonstrated by radiology and computed tomography. The rarity of this pathology made radiological diagnosis difficult