Abstract:

INTRODUCTION: APECED (Autoimmune polyendocrinopathycandidiasis-ectodermal dystrophy) is a rare autosomal recessive disease characterized primarily by sequential immune-mediated destruction of endocrine tissues, chronic oral or mucocutaneous candidiasis and ectodermal disorders, including hypoplasia of dental enamel and delay of tooth eruption. This syndrome may be associated with amelogenesis imperfecta, oral candidiasis and high caries risk. Oral findings are important in the early diagnosis of this syndrome. CASE REPORT: A 12-year-old male patient with APECED syndrome was referred to our clinic with complaints of toothache, lack of teeth and halitosis. Clinical examination of the patient further revealed amelogenesis imperfecta, tooth eruption delay and dental caries associated with poor oral hygiene. Following periodontal, surgical, restorative and endodontic treatments, a removable prosthesis was applied. The patient is still under our observation. CONCLUSION: Oral hygiene education and regular dental check-ups are important since these patients are prone to caries because of enamel hypoplasia. As oral candidal lesions are among the first signs, dentists may have a critical role in the diagnosis of the syndrome. Referral by dentists to an endocrinologist of the young patient with oral candidal lesions may help the early diagnosis of the APECED syndrome.

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