Triple A syndrome is a rare autosomal recessive disorder characterised by alacrimia, achalasia and adrenal failure. It was first reported by Allgrove in 1978 and 100 cases have been reported worldwide. This case report concerns a 24-year-old woman who was referred for evaluation of dysphagia and was finally diagnosed as such a case. A high degree of suspicion enables all the components of this syndrome to be searched for, as early diagnosis can reduce the morbidity and mortality.
MullaneyPBWeatherheadRMillarL, et al.Keratoconjunctivitis sicca associated with achalasia of the cardia, adrenocortical insufficiency and lacrimal gland degeneration: Keratoconjunctivitis sicca secondary to lacrimal gland degeneration may parallel degenerative changes in esophageal and adrenocortical function. Ophthalmology1998; 105: 643–650.
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KinjoSTakemotoMMiyakoK, et al.Two cases of Allgrove syndrome with mutations in the AAAS gene. Endocr J2004; 51: 473–4777.
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RomanSNicolinoMMionF, et al.Triple-a syndrome: a rare etiology of adult achalasia. Dig Dis Sci2005; 50: 440–442.
