Allgrove syndrome associates achalasia, addisonianism, alacrima (OMIM 231550), It is an autosomal recessive disorder recently associated with the AAAS gene coding for the Aladin protein.

The pathophysiology of achalasia in Allgrove’s syndrome remains obscure.

Synopsis

 fibrosis of the intermuscular plane
 myenteric ganglia
 Neuronal NO synthase absent

Etiology

 The triple-A syndrome is caused by mutation in the gene encoding aladin (AAAS) (MIM.605378).

References

 Khelif K, De Laet MH, Chaouachi B, Segers V, Vanderwinden JM. Achalasia of the cardia in Allgrove’s (triple A) syndrome: histopathologic study of 10 cases. Am J Surg Pathol. 2003 May;27(5):667-72. PMID: 12717251