LIPOID PROTEINOSIS IN 2 SISTERS

Lipoid proteinosis was diagnosed in two daughters of a consanguinous m arriage on the basis of genetic, clinical, light microscopic and ultra structural findings. Hyaline material accumulation, thickening of the basal laminae and the resulting typical onion skin phenomenon were obs erved. In addition to the pathognomonic cutaneous mucosal findings, un usual manifestations such as persistence of deciduous teeth (in one ca se), oligodontia and intracerebral calcifications were observed. In on e patient, the intracerebral calcifications caused epileptic seizures.