Ailesel Alopesi ve Otoimmun Poliglanduler Sendrom Tip 3

Year 2010, Volume: 4 Issue: 4, 229 - 235, 01.04.2010

Fatma Gülru Erdoğan Ayça Törel Ergür Münevver Güven Aysel Gürler

Abstract

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.

Keywords

Alopesi areata, otoimmun poliglanduler sendromlar

FAMILIAL ALOPECIA AND AUTOIMMUNE POLYGLANDULAR SYNDROME TYPE 3

Abstract

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

References

• Wasserman D , Guzman-sanchez DA , Scott K , McMichael A: Alopecia areata. International Journal of Dermatology 2007 ;46 :121-131.
• Hawit F, Silverberg N.B : Alopecia areata in children . Cutis . 2008;82:104 -110.
• Kos L , Conlon J : An update on alopecia areata. Current Opinion in Pediatrics. 2009;21 475-480.
• Hordinsky M, Ericson M: Autoimmunity : alopecia areata. J Investig Dermatol Symp Proc 2004; 9:73-78.
• Colombe B.W, Lou C.D , Price V.H: The genetic basis of alopesi areata: HLA associations with patchy alopecia areata versus alope- si totalis and alopecia universalis. J Investig Dermatol Symp Proc. 1999;4:216-219.
• Betterle C , Zancbetta R: Update on autoimmune polyendocrine syndromes(APS). Acta Biomed 2003;74:9-33.
• Aung K, Salmon M: Polyglandular autoimmune syndrome, type 3. http://emedicine.medscape.com/article/124398-overview.
• Obermayer-Straub P , Manns M.P: Autoimmune polyglandular syndromes. Bailliere’s Clinical Gastroenterology. 1998;12:293- 315.
• Majeroni BA, Patel P : Autoimmune polyglandular syndrome, type 2. Am Fam Physician 2007;75:667-670.
• Amerio P , Tracanna M , De Remigis P , Betterle C , Vianale L , Marra M.E , Di Rollo D , Capizzi R , Feliciani C , Tulli A : Vitiligo associated with other autoimmune diseases: polyglandular auto- immune syndrome types 3B+C and 4. Clinical and Experimental Dermatology. 2006;31:746-749.
• Klapproth J-MA , Yang VW: Celiac sprue. http://emedicine.meds- cape.com/article/171805-overview.
• Zawahir S , Safta A , Fasano A : Pediatric celiac disease. Current Opinion in Pediatrics. 2009;21:655-660.
• Thom S , Longo BM , Running A , Ashley J: A guide to successful diagnosis and treatment. The Journal for Nurse Practitioners. 2009: 244-252.
• Evans KE , Malloy AR , Gorard DA . Changing patterns of coeliac serology requests. Alimentary pharmacology&therepeutics.2009;2 9:1137-1142.
• Van den Driesshe A , Eenkhhoorn V , Van Gaal L , De Block C : Type 1 diabetes and autoimmune polyglandular syndrome: a clini- cal review. Neth J Med. 2009;67:376-387.
• Glader B : Megaloblastic Anemias . Nelson Textbook of Pediatrics. Ed. Behrman RE , Kliegman RM , Jenson HB . 17. baskı . U.S.A ,Elsevier Science 2004;1611-1613.
• Carmel R: Reassessment of the relative prevalences of antibodies to gastric parietal cell and to intrinsic factor in patients with perni- cious anemia. Clin Exp Immunol. 1992;89:74-77.
• Suzuki C , Hirai Y , Terui K , Kohsaka A , Akagi T , Suda T . Slowly progressive type1 diabetes mellitus associated with vitili- go vulgaris , chronic thyroiditis , and pernicious anemia. Internal Medicine. 2004;43:1183-1185.
• Shimomura H , Nakase Y , Furuta H , Nishi M , Nakao T , Hanabusa T , Sasaki H , Okamoto K , Furukawa F , Nanjo K : A rare case of autoimmune polyglandular syndrome type3. Diabetes Research and Clinical Practice . 2003;61:103-108 .
• Ugur-Altun B , Arıkan E , Guldiken S , Kara M , Tugrul A : Autoimmune polyglandular syndrome type 3 in monozygotic twins: a case report . Acta Clinica Belgica . 2004;59:225-228.