Triple A (Allgrove) Syndrome

Abstract

Allgrove syndrome is a rare autosomal recessive disorder characterized by classic
triad of Achalasia cardia, Alacremia and ACTH-resistant adrenal insufficiency. Although Addison's
disease is the essential component but various combinations of other major findings are often
seen involving nervous system. Patients generally present with adrenal insufficiency diagnosed
during an inter-current illness. Other clinical features such as alacremia, dysphagia with recurrent
vomiting may precede adrenal insufficiency for some time. Here, we present case of a 5 year-old
boy who presented with complaints of recurrent vomiting and dysphagia. In last admission
tendency towards low blood sugar was noticed and there was some concern about pigmentation
of lips. This paper highlights early features of this syndrome and the importance to include
Allgrove syndrome in the presence of any of two features, progressive dysphagia Alacremia or
symptoms of primary adrenal insufficiency.