Allgrove Syndrome(triple-a Syndrome):a Case Report

Triple-A syndrome, also known as Allgrove syndrome, is an uncommon disorder which is inherited as an autosomal recessive pattern of inheritance, associated with mutations in the AAAS (achalasia–addisonianism–alacrima-syndrome) gene About 100 cases have been described in literature. The three AAA comprises Adrenal insufciency secondary to Adrenocorticotropic hormone (ACTH) resistance, Achalasia cardia, and Alacrimia as well as central and peripheral nervous system involvement. We are reporting a case of a 24 year old diagnosed as triple-A syndrome with ACTH resistant Adrenal insufciency, achalasia cardia, and alacrimia. She had Alicrimia since birth, and at the age of 15, she was diagnosed to have achalasia cardia.She developed signs and of symptoms of Adrenal insufciency at the age of 7 years. Assessment of ACTH, cortisol, ACTH stimulation test conrmed she had adrenal insufciency and physical examination showed she had foot deformity due to muscular atrophy caused by neuropathy.The diagnosis of Allgrove syndrome should be considered in every patient presenting with alacrimia or achalasia cardia.Her condition improved with steroids and articial teardrops. Allgrove's syndrome may be an under diagnosed disorder. High index of suspicion is needed when patients present with such complex symptoms. Diagnosing and timely intervention helps in reducing the morbidity and mortality