Case Report | DOI: https://doi.org/10.31579/2578-8949/JDD/048
Dermatology Department, University Hospital Hassan II, Morocco
*Corresponding Author: Chaouche M, Dermatology Department, University Hospital Hassan II, Morocco
Citation: Chaouche M , Dah Cherif A, Barbach Y, Gallouj S, Mernissi FZ Multiple Nail Candidosis in a Child with APECED Syndrome , Journal of Dermatology and Dermatitis DOI :10.31579/2578-8949/JDD/048
Copyright: © 2019 Chaouche M This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Received: 24 April 2019 | Accepted: 02 May 2019 | Published: 22 May 2019
Keywords: APECED syndrome ; mucocutraneous candidosis ; AIRE gene
The APECED syndrome (autoimmune polyendocrinopathy, candidosis, ectodermal dystrophy) is a rare autosomal recessive disorder that develops in early childhood and results in tissue-specific multiorgan autoimmunity, leading to the hypofunction of multiple glands. Is clinically defined as the presence of at least two components of the classic triad of hypoparathyroidism, adrenal insufficiency, and mucocutaneous candidiasis. We report a case in a child, illustrating the importance of dermatological disorders.
The APECED syndrome (autoimmune polyendocrinopathy, candidosis, ectodermal dystrophy) is a rare condition of autosomal recessive inheritance linked to mutations of the AIRE gene(1 ;2). We report a case in a child, illustrating the importance of dermatological disorders.
A 9-year-old boy, from a second-degree consanguineous marriage, followed in pediatrics for APECED syndrome confirmed by the presence of the mutation of the AIRE gene , with autoimmune hypoparathyroidism, nephrocalcinosis, autoimmune hepatitis and nail dystrophies evolving since three 3 years.
Dermatologic examination revealed pachyonychia with xantonychia, leukonychia, distolateral onycholysis and hyperkeratosis under the nail of the twenty nails. The mycological specimen returned positively to candida albicans. Oesogastroduodenal fibroscopy returned normal. Systemic antimycotic treatment has been proposed.
The APECED syndrome is a rare, serious genetic disease of autosomal recessive inheritance, which must be considered in the face of recurrent cutaneous candidosis and mucosal candidois infections with a characteristic triad consisting of recurrent mucocutaneous candidosis, hypoparathyroidism and autoimmune adrenal insufficiency(3 ;4). The care is difficult, multidisciplinary based on patient education, hormonal supplementation and long-term systemic antimycotic therapy(5).
Knowing the cutaneous disorders of the APECED syndrome allows the dermatologist to diagnose it early, before the appearance of the serious endocrine disorders.
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