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Lipoid proteinosis: case report.
Pediatr Dermatol
June 1989
Department of Dermatology, Hospital St. Jacques, Besançon, France.
Lipoid proteinosis is a rare autosomal recessive condition affecting the majority of organ systems, but predominantly involving skin and mucous membranes. The mucocutaneous infiltration due to accumulation of a hyaline material is positive for both sudanophil and periodic acid-Schiff reagents. This was illustrated in a 14-year-old girl with parental consanguinity, who had classic manifestations.
View Article and Find Full Text PDFThe localization of sudanophil material at dentine, the compact bone and epiphyseal cartilage plate of tibia of rat given beryllium carbonate was examined. Sudanophil material was seen at the boundary parts between dentine and widened predentine, and between widened preosseous matrix and calcified bone, but it was not seen at the area corresponding to the zone of provisional calcification. These facts suggest that the localization of sudanophil material in hard tissue of rat with Be rickets was similar to that in vitamin D deficient-induced rickets.
View Article and Find Full Text PDFSome cytological and histochemical characteristics of skin, the kidney and interrenal tissue were studied in Xenopus laevis adult specimens (1) kept in their natural aquatic environment; (2) gradually exposed to dry conditions under which they were kept one week; and (3) returned from the dry environment to water for 24 hr or a week. In the skin, the most relevant changes are those exhibited by epidermal "flask" cells. These cells are generally rather lean, tall and PAS-positive in "dry" animals and in those 24 hr after replacement in water, whereas in animals placed back in water for a week "flask" cells are often large and faintly stainable.
View Article and Find Full Text PDFThe oral manifestations in 27 patients with Urbach--Wiethe disease (UWD) discovered in Northern Sweden are described. The oral regions most frequently affected are the lips, the back of the tongue, the frenulum of the tongue, the palate and the back wall of the pharynx. The general impression is that older patients usually have more marked manifestations than younger, indicating that the oral lesions may become more severe with increasing age.
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