Alacrimia Revealing Triple A Syndrome: A Case Report

Abstract

Introduction: Allgrove's syndrome is a rare hereditary disease associating alacrimia, achalasia and Addison's disease. Neurological signs are observed in a third of the cases. We report the observation of a 10-year-old child with congenital alacrimia in the context of Allgrove syndrome. Patients and Methods: We report the case of a 10-year-old patient with triple A syndrome. Results: The ophthalmological examination revealed a superficial punctate keratitis with an altered Schirmer's test. The somatic examination found a perioral melanoderma, with amyotrophy of the leg and hollow foot. The explorations performed showed an Addison's syndrome in both children associated with achalasia. Discussion: Congenital alacrimia is the most frequently reported sign, it concerns the basic lacrimal secretion and often also the reflex secretion. Adrenocortical insufficiency is manifested during the first decade of life by severe hypoglycemic crises. Achalasia is revealed between 6 months and 15 years of age by vomiting and dysphagia. Conclusion: In front of any alacrimia of the child or the young adult must evoke a syndrome of Allgrove. A careful search for adrenal insufficiency, achalasia and associated neurological signs is necessary and allows to watch out for complications that could put at risk the vital prognosis.