Allgrove Syndrome: A Case Report

A Soltani; M Arab Ameri; SH Hasani Ranjbar

International Journal of Endocrinology and Metabolism

Research Institute for Endocrine Sciences

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Allgrove Syndrome: A Case Report

Author(s):

A Soltani^1,*,

M Arab Ameri²,

SH Hasani Ranjbar²

1Endocrinology and Metabolism Research Center, Tehran University of Medical Sciences, emrc@sina.tums.ac.ir, I.R Iran

2Endocrinology and Metabolism Research Center, Tehran University of Medical Sciences, I.R Iran

International Journal of Endocrinology and Metabolism:Vol. 5, issue 4; 160-163

Article type:Case Report

Received:Jun 09, 2007

Accepted:Jul 17, 2007

How to Cite:A SoltaniM Arab AmeriSH Hasani RanjbarAllgrove Syndrome: A Case Report.Int J Endocrinol Metab.5(4):160-163.

Abstract

Allgrove syndrome (triple A syndrome) is an autosomal recessive disorder characterized by achalasia, alacrima and adrenocorticotropic hor-mone (ACTH) resistant adrenal insufficiency. It is a multisystem disease and in addition to cardinal manifestations, associated features es-pecially neurologic problems, must be detected and treated. In this case report, we report a 17 year-old boy diagnosed as having Allgrove syndrome with predominant symptoms of achalasia and additional features consisting of short neck, long eye lashes, unexplained fever and chills, reduced visual acuity because of amblyopia, thenar and hypothenar atrophy and abnormal opposition of fingers. If necessary screening with stimulatory tests in patients with unexplained features such as long eye lashes, short neck, muscle atrophy, na-sal speech, skin and neurologic abnormalities and hyperkeratosis, should be recommended.

Keywords

Adrenocorticotropin insensitivity syndrome.

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