Hakan KARDEŞ, Zühal ÖRNEK, Coşkun EKEMEN, Ahmet GİRGEÇ

Tekrarlayan Lokalize Döküntü ve Halsizlikle Başvuran Ailesel Akdeniz Ateşi Olgu Sunumu

Otozomal resesif kalıtılan Ailesel Akdeniz Ateşi tekrarlayan ateş, döküntü, artrit, artralji, karın ağrısı gibi klinik bulgularla karakterizedir. Birçok sistemi tutabilen FMF’nin en önemli komplikasyonu böbrek tutulumuna bağlı amiloidoz gelişimidir. Tedavide kullanılan temel ilaç ise kolşisindir. Bu makalede çocuk hastalıkları polikliniğine tekrarlayan tek taraflı lokalize ayak bileğinde ödem, kızarıklık ve döküntü yakınmaları ile başvuran ve takibinde klinik ve genetik tetkikler sonucunda FMF tanısı alan bir olgu sunulmuştur. Tekrarlayan ateş, karın ağrısı, artrit veya artralji ya da izole erizipel benzeri döküntüsü olan çocuklarda FMF ön tanıda mutlaka akla gelmelidir. Bu sayede çocuklarda erken amiloidoz gelişimi ve FMF’e bağlı komplikasyonlar da engellenmiş olacaktır.

Report of Familial Mediterranean Fever with Repeating Localized Rash and Weakness

Familial Mediterranean Fever (FMF), which is inherited as autosomal recessive, is characterized by recurrent fever, rash, arthritis, arthralgia, and abdominal pain. The most important complication of FMF, which can affect many systems, is amyloidosis development due to kidney involvement. The main drug used in the treatment is colchicine. In this article, a case which arrived at the pediatric clinic with recurrent unilateral localized ankle complaints of edema, erythema together with rash and which, as a result of clinical and genetic tests, was diagnosed as FMF is examined. Symptoms observed at the preliminary diagnosis such as recurrent fever, abdominal pain, arthritis or arthralgia or isolated erysipelas should definitely remind pediatricians FMF. In this way, early amyloidosis development and complications due to FMF will be prevented in children.

PDF

___

1. Alghamdi M. Familial Mediterranean fever, review of the literature. Clin Rheumatol. 2017;36(8):1707-13.
2. Sönmez HE, Batu ED, Özen S. Familial Mediterranean fever: Current perspectives. J Inflamm Res. 2016;9:13-20.
3. Padeh S, Berkun Y. Familial Mediterranean fever. Curr Opin Rheumatol. 2016;28(5):523-9.
4. Ben-Chetrit E, Touitou I. The impact of MEFV gene identification on FMF: An appraisal after 15 years. Clin Exp Rheumatol 2012;30:S3-S6.
5. Kallinich T, Wittkowski H, Keitzer R, Roth J, Foell D. Neutrophil-derived S100A12 as novel biomarker of inflammation in familial Mediterranean fever. Ann Rheum Dis 2010;69:677-682.
6. Tunca M, Akar S, Onen F, Ozdogan H, Kasapcopur O, Yalcinkaya F, et al. Familial Mediterranean fever (FMF) in Turkey: results of a nationwide multicenter study. Medicine (Baltimore) 2005;84:1-11.
7. Ben-Chetrit E, Touitou I. Familial mediterranean Fever in the world. Arthritis Rheum. 2009;61:1447-53.
8. Önen F. Ailevi Akdeniz ateşi. Rheumatol Int. 2006;26:489- 96.
9. Brik R, Shinawi M, Kasinetz L, Gershoni-Baruch R. The musculoskeletal manifestations of familial Mediterranean fever in children genetically diagnosed with the disease. Arthritis Rheum. 2001;44:1416-9.
10. Jarjour RA, Dodaki R. Arthritis patterns in familial Mediterranean fever patients and association with M694V mutation. Mol Biol Rep. 2011;38:2033-6.
11. Ince E, Cakar N, Tekin M, Kendirli T, Ozkaya N, Akar N, et al. Arthritis in children with familial Mediterranean fever. Rheumatol Int. 2002;21:213-7.
12. Üzüm Ö ve ark. Familial Mediterranean fever and prolonged febrile myalgia syndrome: Case report. İzmir Dr. Behçet Uz Çocuk Hast Dergisi. 2017; 7(2):164-166
13. Barzilai A, Langevitz P, Goldberg I, Kopolovic J, Livneh A, Pras M, et al. Erysipelas-like erythema of familial Mediterranean fever: Clinicopathologic correlation. J Am Acad Dermatol. 2000;42:791-5.
14. Petty RE, Cassidy JT. Textbook of pediatric rheumatology. Philadelphia: Saunders Elsevier; 2011
15. Lidar M, Doron A, Barzilai A, et al. Erysipelas-like erythema as the presenting feature of familial Mediterranean fever. J Eur Acad Dermatol Venereol 2013; 27: 912.
16. Pras M. Familial Mediterranean fever: From the clinical syndrome to the cloning of the pyrin gene. Scand J Rheumatol. 1998;27:92-7.