Fatma Gülru ERDOĞAN, Ayça Törel ERGÜR, Münevver GÜVEN, Aysel GÜRLER

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Ailesel Alopesi ve Otoimmun Poliglanduler Sendrom Tip 3

Dermatoloji polikliniklerinde sıkça görülen alopesi areatanın genetik yatkınlık ve çevresel tetikleyici bir faktör ile ortaya çıkan organ spesifi k otoimmun bir hastalık olduğu ve sıklıkla diğer otoimmun hastalıklarla ilişkili olabildiği bilinmektedir. Alopesinin diğer otoimmun hastalıklarla birlikteliğinde otoimmun poliglanduler sendromlar tanımlanmaktadır. Otoimmun poliglanduler sendromlar günümüzde OPS tip 1, 2, 3, 4 olarak 4 ana tipe ayrılmakta olup, alopesi her tipte bulunabilmekle beraber en sık OPS tip 3’te görülmektedir. OPS tip 3 tanısı için otoimmun tiroid hastalığına ilaveten Addison hastalığı ve/veya hipoparatiroidizm dışında bir otoimmun hastalığının mevcudiyeti gerekmektedir. 2001 yılında Beterle OPS tip 3’ü 4 subgruba (tip 3A, 3B, 3C, 3D) ayırmış olup otoimmun tiroid hastalığına ilaveten alopesinin bulunması OPS tip 3C olarak sınıfl andırılmıştır.Burada alopesi areata tanısıyla kliniğimize başvurmuş 6 yaşında erkek hasta ve 5 yaşındayken alopesi universalis tanısı almış ağabeyi zaman içinde poliglandüler tutulum geliştirmeleri nedeniyle, alopesi areataya eşlik edebilecek otoimmun hastalıklar ve genetik incelemeler vurgulanmak istenmiştir.
Anahtar Kelimeler:

Alopesi areata, otoimmun poliglanduler sendromlar

FAMILIAL ALOPECIA AND AUTOIMMUNE POLYGLANDULAR SYNDROME TYPE 3

Alopecia areata, which is frequently seen in dermatology policlinics, is known to be an organ specifi c autoimmune disease appearing with a genetic tendency and environmental triggering factors and is often associated with other organ specifi c autoimmune diseases. Autoimmune polyglandular syndromes are defi ned for the association of alopecia with other autoimmune diseases. Autoimmune polyglandular syndromes (APS) are divided into 4 major types as type 1, 2, 3, and 4. Alopecia appears in all types, especially in type 3 of APS. For the diagnosis of APS type 3, besides the presence of autoimmune thyroid disease, another autoimmune disease except Addison’s disease or hypoparathyroidism is required. In 2001, Betterle subdivides APS type 3 into four subgroups (Type 3A, 3B, 3C, and 3D) and coincidence of autoimmune thyroid disease with alopecia is classifi ed as type 3C.Here, 6-year-old male patient with the diagnosis of alopecia areata and his brother diagnosed as alopecia universalis when he was 5-year-old eventually had polyglandular involvement are presented to emphasize associated autoimmune diseases and genetic studies
Keywords:

Alopecia areata, autoimmune polyglandular syndromes,

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Türkiye Çocuk Hastalıkları Dergisi-Cover
  • ISSN: 1307-4490
  • Başlangıç: 2007
  • Yayıncı: -
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Ailesel Alopesi ve Otoimmun Poliglanduler Sendrom Tip 3

Fatma Gülru ERDOĞAN, Ayça Törel ERGÜR, Münevver GÜVEN, Aysel GÜRLER