title: [Autoimmune polyendocrinopathy and chronic mucocutaneous candidiasis].
abstract: The authors report the case of a child who, at the age of 18 months showed signs of hypoparathyroidism together with gastrointestinal, then buccal, then ungual candidiasis. Acute adrenal failure occurred when he was 5 1/2 years' old. At the age of 10, the patient developed alopecia areata and interstitial keratitis. Immunological investigations yielded normal results, except that serum was weakly positive for anti-adrenal antibodies at 1/10th. The mucosal and ungual candidiasis infection was cured by ketoconazole, and the various endocrine abnormalities were corrected with the appropriate replacement therapies. This case prompted the authors to review the candidiasis/"polyglandular autoimmune disease" association. Whitaker's triad consists of candidiasis, hypoparathyroidism and chronic renal failure, 2 or these 3 elements being sufficient to make the diagnosis. Numerous other associations have been described; they are presented here in table form in descending order of frequency, with candidiasis/hypoparathyroidism coming on top of the list (70 p. 100). The fairly constant chronological order in which these different pathologies appear is one of the peculiarities of the syndrome: candidiasis often precedes hypoparathyroidism and adrenal insufficiency. Alopecia areata does not seem to be frequent, but its true incidence is difficult to quantify since lesions of the scalp and/or skin appendages are poorly documented in the literature. Alopecia and keratopathy seem to be of autoimmune origin. Mucocutaneous candidiasis too is specific, the mucosae and nails being constantly involved. This type of candidiasis does not exist in other forms of hypoparathyroidism. Chronic mucocutaneous candidiasis is found in many different diseases and is due to immunodeficiency against Candida spp.(ABSTRACT TRUNCATED AT 250 WORDS)
authors.affiliation: Unité de Dermatologie, Hôpital Necker-Enfants Malades, Paris.
authors.forename: C
authors.initials: C
authors.lastname: Delambre
authors.forename: D
authors.initials: D
authors.lastname: Teillac
authors.forename: R
authors.initials: R
authors.lastname: Brauner
authors.forename: Y
authors.initials: Y
authors.lastname: de Prost
mesh_terms: D001327:Autoimmune Diseases / Q000150:complications*; D002177:Candidiasis / Q000150:complications*; D002178:Candidiasis, Chronic Mucocutaneous / Q000150:complications*; D004700:Endocrine System Diseases / Q000150:complications*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant
vernacular_title: Polyendocrinopathie auto-immune et candidose mucocutanée chronique.