Premature Loss of Permanent Teeth in Allgrove (4A) Syndrome in Two Related Families.

[triple a syndrome]

Allgrove syndrome is a rare autosomal recessive condition characterized by adrenal insufficiency , achalasia , alacrima and occasionally autonomic disturbances . Mutations in the AAAS gene , on chromosome 12 q 13 have been implicated as a cause of this disorder .We present various manifestations of this syndrome in two related families each with two affected siblings in which several members had symptoms including reduced tear production , mild developmental delay , achalasia , neurological disturbances and also premature loss of permanent teeth in two of them .The importance of this report is dental involvement (loss of permanent teeth ) in Allgrove syndrome that has not been reported in literature .