Primary gelatinous drop-like corneal dystrophy in a white woman. A pathologic, ultrastructural, and immunohistochemical study

Détails

ID Serval
serval:BIB_16D7F0EC2001
Type
Article: article d'un périodique ou d'un magazine.
Sous-type
Etude de cas (case report): rapporte une observation et la commente brièvement.
Collection
Publications
Institution
Titre
Primary gelatinous drop-like corneal dystrophy in a white woman. A pathologic, ultrastructural, and immunohistochemical study
Périodique
Cornea
Auteur⸱e⸱s
Buchi  E. R., Daicker  B., Uffer  S., Gudat  F.
ISSN
0277-3740 (Print)
Statut éditorial
Publié
Date de publication
03/1994
Volume
13
Numéro
2
Pages
190-4
Notes
Case Reports
Journal Article --- Old month value: Mar
Résumé
A white female with primary gelatinous drop-like corneal dystrophy (PGDD) was followed from the ages of 8 to 37 years. During this time, she underwent repeated lamellar and penetrating keratoplasties on both sides due to relapsing bilateral corneal opacifications. The diagnosis of PGDD was based on the histology of the corneal specimens obtained at 10 and 36 years of age, as well as on the characteristic clinical appearance and course. Electron microscopy showed typical amyloid fibrils. Immunohistochemical staining was mildly positive for amyloid AL (light chain), but negative for amyloid AA, AF, AB, and keratin. Thus, the precursor protein of the amyloid deposits in PGDD might derive from immunoglobulins, e.g., from the tear film. However, the precursor protein might derive from a different origin altogether. In that case, the positive staining for amyloid AL would translate nonspecific absorption of fragments of light chains.
Mots-clé
Adult Amyloidosis/*pathology Corneal Diseases/*pathology Corneal Dystrophies, Hereditary/*pathology Female Follow-Up Studies Humans Keratoplasty, Penetrating Longitudinal Studies Reoperation
Pubmed
Web of science
Création de la notice
28/01/2008 13:02
Dernière modification de la notice
20/08/2019 12:46
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