1/5. Periodontal lesions in lipoid proteinosis.Lipoid proteinosis is a rare autosomal recessive heritable disease. Clinical features are hoarseness starting in infancy and the formation of yellowish papules and plaques on the skin and mucous membranes of the nose, oral cavity, pharynx and larynx. light and electron microscopy demonstrates a subepithelial accumulation of PAS-positive (hyaline) material around blood vessels and in the dermis. This case is unusual in that the gingiva is also involved in the lipoid proteinosis.- - - - - - - - - - ranking = 1keywords = oral cavity, cavity (Clic here for more details about this article) |
2/5. Lipoid proteinosis.Lipoid proteinosis (Urbach-Wiethe disease) is a rare, recessively inherited disorder that is characterized by the deposition of hyaline-like material in the skin, oral cavity, and other tissues. It usually appears in infancy with hoarseness. We report a case of lipoid proteinosis in a 10-year-old boy that demonstrates the characteristic clinical, histologic, and ultramicroscopic features of this disease.- - - - - - - - - - ranking = 1keywords = oral cavity, cavity (Clic here for more details about this article) |
3/5. Hyalinosis cutis et mucosae. review with a case report.Hyalinosis cutis et mucosae (lipoid proteinosis) is a rare disorder characterized by hyaline and lipid deposits in the skin and mucous membrane of the oral cavity, upper respiratory tract, and internal organs. It usually appears in infancy with hoarseness. This disorder is particularly interesting because of its rarity and wide range of clinical manifestations. We report a case that demonstrated manifestations of lipoid proteinosis.- - - - - - - - - - ranking = 1keywords = oral cavity, cavity (Clic here for more details about this article) |
4/5. Lipoid proteinosis: clinical, histologic, and ultrastructural investigations.The case of a 12-year-old boy with lipoid proteinosis is reported. physical examination revealed long-standing varicella-like scars and areas of hyperpigmentation on the face and upper limbs with no evidence of photosensitivity, hoarseness, small papules along the free margins of eyelids, tongue firmness with short frenulum, and widespread papular lesions of the oral cavity. Histologic and ultrastructural examination revealed the characteristic skin changes: pink, hyaline-like, strongly periodic acid-Schiff-positive material in the dermis, surrounding blood vessels, and sweat glands; thin (30 to 35 nm) collagen fibrils interspersed in abundant amorphous material; blood vessels surrounded by thickened, multilayered basement membranes, in which layers of typical, homogeneous basement membrane material were alternating with electronlucent areas filled by various amounts of thin, cross-striated fibrils, arranged perpendicularly. These findings are of great interest since they show a complex relationship between type IV and type III-like collagen components.- - - - - - - - - - ranking = 1keywords = oral cavity, cavity (Clic here for more details about this article) |
5/5. Lipoid proteinosis of the oral mucosa: case report and review of the literature.We describe a 37-year-old woman who presented with progressive mouth dryness. physical examination revealed long-standing plaques on the face and upper limbs, papular lesions of the oral cavity and tongue firmness. A lower lip biopsy was performed. light microscopy demonstrated accumulation of PAS-positive material around blood vessels, capillaries and salivary gland canaliculi as well as focally massive hyaline deposits in the submucosa. immunohistochemistry revealed widespread presence of type IV collagen in the hyaline material and around thickened blood vessels. laminin immunoreactivity was particularly strong at thickened basement membranes. The above findings were compatible with lipoid proteinosis, which is likely to involve primary perturbation of collagen metabolism and production of glycoproteins.- - - - - - - - - - ranking = 1keywords = oral cavity, cavity (Clic here for more details about this article) |