Background: Familial candidiasis endocrinopathy is a hereditary disease variably associated with i) a dysfunction of the parthyroid, adrenal, thyroid, pituitary or ovarian glands as well as the pancreas (diabetes), liver (hepatitis) and gastric wall cells (Biermer's disease); ii) chronic mucocutaneous candidiasis; iii) autoimmune manifestations: chronic keratitis, pelade, vitiligo, hemolytic anemia.
Case reports: We cared for three pediatric patients with chronic mucocutaneous candidiasis associated with an autoimmune polyendocrinopathy. There was a familial context in all cases with an autosomal dominant inheritance in the first case and an autosomal recessive inheritance in the two others who were siblings. In all three cases, the chronic mucocutaneous candidiasis began before the age of 5 years and had preceded the development of the endocrinopathy. Fluconazole was effective in all three cases.
Discussion: Chronic mucocutaneous candidiasis may appear as the inaugural symptom of an autoimmune polyendocrinopathy and should lead to a familial study with an andocrinology work-up to determine any clinical manifestations and biological evidence of autoimmunity. Fluconazole does not have marketing approval for children, but provides good control of the fungal infection, particularly in the ungueal localization, and is well tolerated.