Triple A syndrome (Allgrove syndrome) - A journey from clinical symptoms to a syndrome

Prakarti Yadav; Deepak Kumar; Gopal K Bohra; Mahendra K Garg

doi:10.4103/jfmpc.jfmpc_237_20

Triple A syndrome (Allgrove syndrome) - A journey from clinical symptoms to a syndrome

J Family Med Prim Care. 2020 May 31;9(5):2531-2534. doi: 10.4103/jfmpc.jfmpc_237_20. eCollection 2020 May.

Authors

Prakarti Yadav¹, Deepak Kumar¹, Gopal K Bohra¹, Mahendra K Garg¹

Affiliation

¹ Department of Medicine, All India Institutes of Medical Sciences Jodhpur, Rajasthan, India.

Abstract

Triple A syndrome (Allgrove syndrome) is characterized by a triad of specific features, namely, alacrimia, adrenal insufficiency, and achalasia cardia. It is a rare autosomal recessive disorder. In the present study, an 18-year-old boy was presented with complaints of decreased tears, darkening of the skin, difficulty in walking and standing up from sitting position, and difficulty in swallowing liquids. Adrenal insufficiency, alacrimia, achalasia, and neurological manifestations were confirmed with relevant laboratory investigations. His condition improved with steroids and artificial teardrops. However, a vigilant eye of the clinician for clinical clues of syndromic manifestation will help in early diagnosis and proper management.

Keywords: Achalasia cardia; Adrenal insufficiency; Alacrimia; Allgrove's syndrome; Triple A syndrome.

Publication types

Case Reports