Familial occurrence of oral submucous fibrosis: report of eight families from northern Kerala, south India.
BACKGROUND
Oral submucous fibrosis (OSF) is an archetype of pathological fibrosis due to possibly its presence for centuries amongst people of Indian ethnic extraction. The WHO criteria for a precancerous condition accords well with this disease. The fact that the disease has been reported in at least few subjects who do not practice the habit of areca nut chewing and the inability to prove a dose-effect relationship in almost all cases, the question arose whether there is a predisposition for the disease.


METHODS
A house- to-house survey was carried out in northern Kerala, South India, for the detection of early and advanced OSF from June 2003 through May 2004. An analysis of the family pedigrees of all patients with a positive history of fibrosis has been carried out.


RESULTS
Preliminary analysis of the family survey revealed that OSF tends to aggregate in families. The pedigrees of eight (8) such families presented here and the concept of OSF having a genetic predisposition to its occurrence revisited.


CONCLUSIONS
The view that OSF having a family predilection, at least in a proportion of cases, and its tendency for familial linkage seems to be apparent by this study. This rather denovo observation of a trend relationship between OSF risk and the number of putative high-risk genotypes should be separately evaluated for the collagen metabolizing and cross-linking pathways. Establishing familial linkage of the disease seems to be an initial step in deciphering further the molecular pathways involved in its causation and biologic progression.
Rajendran
R
R
Dept. Oral Pathology, Govt. Dental College Trivandrum, India. rrajendran_r@yahoo.co.in
Vidyarani
D000328:Adult; D017668:Age of Onset; D001114:Areca; D002294:Carcinoma, Squamous Cell / Q000150:complications; D002648:Child; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D007194:India; D008297:Male; D008875:Middle Aged; D009062:Mouth Neoplasms / Q000150:complications; D009914:Oral Submucous Fibrosis / Q000235:genetics*; D010375:Pedigree; D011446:Prospective Studies