Typical Radiological Findings of Lipoid Proteinosis of Urbach and Wiethe

Abstract

Lipoid proteinosis is a rare autosomal recessive dermatosis, which can be congenital or have onset in infancy. It is characterized by the progressive deposition of an amorphous hyaline substance with a glycoprotein constitution in the skin and mucous membranes and presents as papular lesions that can aggregate forming plaques with a yellowish discoloration. We present a case of lipoid proteinosis in a seven-year-old boy where characteristic calcifications in the medial temporal lobe helped in early initiation of treatment.