APECED SYNDROME (FAMILY OBSERVATION)

Introduction: APECED (Autoimmune Polyendocrinopathy Candidiasis Ectodermal Dystrophy) is an autosomal recessive disease characterized by the association of autoimmune endocrine involvement, mucocutaneous candidiasis and ectodermal tissue involvement. Observation: We report case 2 brothers from a 2nd degree consanguineous marriage with history deaths in siblings for whom diagnosis syndrome was retained on clinical biological criteria (genetic study progress).The eldest had candidous onychomycosis hands feet, generalized candidiasis, profound hypocalcemia tetany crisis, hepatitis, Biermers anemia ADDISONs disease. Meanwhile youngest onychomycosis, vitiligo, atrophy tongue, alopecia adrenalinsufficiency. Discussion:Autoimmune polyglandular type 1 (APS-1) classically at least two three components Whitakers triad: chronic (CMC), hypoparathyroidism, adrenal insufficiency. If sibling affected, only one component triad necessary to make diagnosis. Conclusion: This requires multidisciplinary approach, without forgetting importance genetic counseling this