In which condition mucocutaneous candidiasis is an important clinical feature?
Ans. A. Auto immune hypoparathyroidWhen immune dysfunction affects two or more endocrine glands and other non-endocrine immune disorders are present, the polyglandular autoimmune (PGA) syndromes should be considered. The PGA syndromes are classified as two main types: the type I syndrome starts in childhood and is characterized by mucocutaneous candidiasis, hypoparathyroidism, and adrenal insufficiency; the type II, or Schmidt, syndrome is more likely to present in adults and most commonly includes adrenal insufficiency, thyroiditis, or type 1 diabetes mellitus. THE type III syndrome is heterogeneous and may consist of autoimmune thyroid disease along with a variety of other autoimmune endocrine disorders.Features of Polyglandular Autoimmune (PGA) SyndromesPGA IPGA IIEpidemiologyAutosomal recessivePolygenic inheritanceMutations in APECED geneHLA-DR3 and HLA-DR4 associatedChildhood onsetAdult onsetEqual male: female ratioFemale predominanceDisease AssociationsMucocutaneous candidiasisAdrenal insufficiencyHypoparathyroidismHypothyroidismAdrenal insufficiencyGraves' diseaseHypogonadismType 1 diabetesAlopeciaHypogonadismHypothyroidismHypophysitisDental enamel hypoplasiaMyasthenia gravisMalabsorptionVitiligoChronic active hepatitisAlopeciaVitiligoPernicious anemiaPernicious anemiaCeliac disease
Auto immune hypoparathyroid
Digeorge syndrome
Pseudo hypoparathyroid
Post-surgical hypoparathyroid
Autoimmune Skin Disorders