ABSTRACT
Two XP patients are presented. A corneal perforation in the left eye of the first patient necessitated an at-random transplantation à chaud. The graft was remarkably well tolerated, which is possibly explained by UV-light-induced suppression of the cellular immune response in the patient. The right eye of this patient and both eyes of the second patient had a keratoconus. Keratoconus in XP may be the result of disturbances in the cell differentiation and the function of epithelial cells and keratocytes, due to UV-light-induced deficient DNA repair synthesis.
Subject(s)
Keratoconus/etiology , Xeroderma Pigmentosum/complications , Adult , Child , Corneal Diseases/pathology , Corneal Diseases/surgery , Corneal Transplantation , Humans , Keratoconus/pathology , Keratoconus/surgery , Male , Xeroderma Pigmentosum/pathologyABSTRACT
Clinicopathologic case report of a boy aged 17 with a ring melanoma of the iris with endothelial overgrowth and glaucoma, with reference to the first case of the iridocorneal endothelial syndrome reported from the same laboratory by Rochat & Mulder in 1924. When a melanoma spreads around the globe within the ciliary body, or around the angle of the anterior chamber, or diffusely over the surface of the iris, it is called a ring melanoma (5).
Subject(s)
Corneal Diseases/pathology , Iris Diseases/pathology , Melanoma/pathology , Uveal Neoplasms/pathology , Adolescent , Endothelium/pathology , Glaucoma/complications , Humans , Iris/pathology , Male , Melanoma/complications , Uveal Neoplasms/complicationsABSTRACT
The clinical history as well as the general and ophthalmic pathology of a woman with the Meretoja type of hereditary systemic amyloidosis and lattice dystrophy of the cornea is described. Extraocularly, amyloid could be demonstrated in all the arterial walls, the peripheral nerves and in the glomeruli; intraocularly, amyloid was seen in the cornea and the sclera.
Subject(s)
Amyloidosis/pathology , Corneal Dystrophies, Hereditary/pathology , Eye/pathology , Aged , Amyloidosis/genetics , Cornea/pathology , Corneal Dystrophies, Hereditary/genetics , Female , Humans , Muscular Atrophy/pathology , Sclera/pathology , SyndromeABSTRACT
Blodi (1957) and François (1958) recognized this syndrome as a distinct entity differing from the mandibulo-facial dysostosis (Franceschetti's syndrome). Although there are approximately 60 cases reported in the literature, only very few cases have been obtained for histological study. A report of a case with ophthalmopathological examination is given.
