Urbach-Wiethe disease/lipoidproteinosis.

R Maia, L Teixeira, J Drago


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.

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