Report of Familial Mediterranean Fever with Repeating Localized Rash and Weakness

Yıl 2020, Cilt: 4 Sayı: 1, 34 - 37, 30.04.2020

Coşkun Ekemen , Ahmet Girgeç , Hakan Kardeş , Zühal Örnek

Öz

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

Anahtar Kelimeler

FMF, Familial Mediterranean Fever, Arthritis, Rash

Tekrarlayan Lokalize Döküntü ve Halsizlikle Başvuran Ailesel Akdeniz Ateşi Olgu Sunumu

Öz

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

Anahtar Kelimeler

FMF, ailesel akdeniz ateşi, artrit, döküntü

Kaynakça

• 1. Önen F. Ailevi Akdeniz ateşi. Rheumatol Int. 2006; 26 : 489–96
• 2. Goldfinger SE. Colchicine for familial Mediterranean fever. N Engl J Med 1972;287:1302.
• 3. Sohar E, Gafni J, Pras M, Heller H. Familial Mediterranean fever. A survey of 470 cases and review of the literature. Am J Med. 1967;43:227–53
• 4. The International FMF Consortium. Ancient missense mutations in a new member of the RoRet gene family are likely to cause familial Mediterranean fever. Cell. 1997;90:797–807.
• 5. Ben-Chetrit E, Touitou I. Familial mediterranean Fever in the world. Arthritis Rheum. 2009;61:1447–53.
• 6. Samuels J, Aksentijevich I, Torosyan Y, Centola M, Deng Z, Sood R, et al. Familial Mediterranean fever at the millennium. Clinical spectrum, ancient mutations, and a survey of 100 American referrals to the National Institutes of Health. Medicine(Baltimore) 1998;77(4):268–97.
• 7. 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.
• 8. 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.
• 9. Jarjour RA, Dodaki R. Arthritis patterns in familial Mediterranean fever patients and association with M694V mutation. Mol Biol Rep. 2011;38:2033–6.
• 10. 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.
• 11. 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
• 12. Ceylan Ö, Ceylan Y. Intermittent right bundle branch block in a child with familial mediterranean fever. Cardiol J. 2018;25(6):753-755.
• 13. Barzilai A, Langevitz P, Goldberg I, Kopolovic J, Livneh A, Pras M, et al. Erysipelas-like erythema of familial Mediterranean fever: clinicopatho logic 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, vd. Erysipelas-like erythema as the presenting feature of familial Mediterranean fever J Eur Acad DermatolVenereol 2013; 27: 912.
• 16. Keskindemirci G ve ark. Çocuklarda Lenfadenopatinin Nadir Bir Nedeni: Otoinflamatuvar Hastalık - Ailevi Akdeniz Ateşi, Turkish J Pediatr Dis ,2016; 4: 274-276
• 17. Pras M. Familial Mediterranean fever: from the clinical syndrome to the cloning of the pyrin gene. Scand J Rheumatol. 1998;27:92–7.
• 18. Dinarello CA, Wolff SM, Goldfinger SE, Dale DC, Alling DW. Colchicine therapy for familial mediterranean fever. A double-blind trial. N Engl J Med. 1974;291:934–7.
• 19. Zemer D, Revach M, Pras M, Modan B, Schor S, Sohar E, et al. A controlled trial of colchicine in preventing attacks of familial mediterranean fever. N Engl J Med.1974;291:9324.
• 20. Goldstein RC, Schwabe AD. Prophylactic colchicine therapy in familial Mediterranean fever. A controlled, double-blind study. Ann Intern Med. 1974;81:792–4.
• 21. Erken E, Ozer HT, Bozkurt B, Gunesacar R, Erken EG, Dinkci S. Early suppression of familial Mediterranean fever attacks by single medium dose methyl-prednisolone infusion. Joint Bone Spine. 2008;75:370–2.
• 22. Meinzer U, Quartier P, Alexandra JF, Hentgen V, Retornaz F, Kone-Paut I. Interleukin-1 targeting drugs in familial Mediterranean fever: a case series and a review of the literature. Semin Arthritis Rheum. 2011;41:265–71.