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

Ailesel alopesi ve otoimmun poliglanduler sendrom tip3

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 sınıflandırılmıştır. 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.

Familial alopecia and autoimmune polyglandular syndrome type 3

Alopecia areata, which is frequently seen in dermatology policlinics, is known to be an organ specifi cautoimmune 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.

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