PMID: 9557893Apr 29, 1998Paper

Retinal changes and tumorigenesis in Ramon syndrome: follow-up of a Brazilian family

American Journal of Medical Genetics
J M de Pina-NetoJ F Colafêmina

Abstract

We report on the clinical evolution of the Brazilian family with Ramon syndrome described by de Pina-Neto et al. [1986, Am J Med Genet 25:441-443]. Three members (patients IV-2, IV-18, and IV-19) have developed pigmentary changes in the retina and paleness of the optic disk. Patient IV-18 also has developed giant hypertrophy of the labia minora that, when examined histopathologically, was found to be due to neoplastic fibroblast and epithelial proliferation caused by a fibromatous process similar to that reported in the gingivae of the patients with this syndrome. Audiologic function of patient IV-2 was normal, and no skin lesions were detected. The articular signs and symptoms show that the affected relatives developed rheumatoid arthritis, which is currently inactive in patient IV-18, whereas patient IV-2 did not develop these alterations.

References

Oct 1, 1967·Oral Surgery, Oral Medicine, and Oral Pathology·Y RamonJ J Bubis

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Citations

May 5, 2006·Journal of Periodontology·Ricardo D Coletta, Edgard Graner
Feb 20, 2007·Journal of Oral and Maxillofacial Surgery : Official Journal of the American Association of Oral and Maxillofacial Surgeons·Edgard Carvalho SilvaTainah Couto Vieira
Dec 18, 2001·American Journal of Medical Genetics·B Parkin, C Law

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