Lipoid proteinosis in 2 sisters
Oezarmağan, G.; Baykal, C.; Gürsoy, E.O.; Yilmazer, S.; Büyükbabani, N.; Coban, O.
Der Hautarzt; Zeitschrift für Dermatologie Venerologie und verwandte Gebiete 44(5): 315-318
1993
ISSN/ISBN: 0017-8470 PMID: 7686542 Document Number: 412263
Lipoid proteinosis was diagnosed in two daughters of a consanguinous marriage on the basis of genetic, clinical, light microscopic and ultrastructural findings. Hyaline material accumulation, thickening of the basal laminae and the resulting typical onion skin phenomenon were observed. In addition to the pathognomonic cutaneous mucosal findings, unusual manifestations such as persistence of deciduous teeth (in one case), oligodontia and intracerebral calcifications were observed. In one patient, the intracerebral calcifications caused epileptic seizures.