Necla BUYAN, Oğuz SÖYLEMEZOĞLU, Kibriya FİDAN, Davut BOZKAYA, Sebahat Ağladıoğlu YILMAZ

Ailevi Akdeniz Ateşi zemininde gelişen sakroileit ve subkorneal püstüler dermatoz birlikteliği

Ailevi Akdeniz Âteşi (AAA), otozomal resesif kalıtılan, kendini sınırlayan tek- . rarlayan ateş, karın, göğüs ve eklem ağrısıyla karakterize nedeni belli olmayan bir hastalıktır. Eklem hastalığı; karın ağrısından sonra ortaya çıkan en sık ikinci semptomdur. Subkorneal Püstüler Dermatoz (SPD), kronik, tekrarlayan püstüler lezyonlarla karakterize iyi huylu bir hastalıktır. Nedeni belli olmayan bu hastalık sıklıkla 40 yas üstü kadınlarda görülmesine rağmen, nadiren çocuklarda da tanımlanmıştır. Bu yazıda, çocukluk yas grubunda nadir görülen SPD'un FMF ve sakroileit ile birlikteliği olan 9 yaşındaki bir çocuk olgusu sunulacaktır.

Juvendle subcorneal pustular dermatosds in a case of Familial Mediterranean Fever and Sacroiliitis

Familial Mediterranean fever (FMF) is an autosomal recessive disease, characterized by recurrent and selflimited attacks of fever, usually accompanied by polyserositis. The articular disease is the second most common/manifestation after abdominal pain. The arthritis of FMF most frequently affecting the large joints of the lower extremities. Sacroiliitis due to FMF is very rare in children. Subcorneal pustular dermatosis (SPD), characterized by chronic, relapsing, pustular lesions. Although it usually occurs in middle-aged women, it is occasionally seen in childhood. There are various cutaneous manifestations of FMF but SPD and FMF association has been not previously described. In this report, we describe a 9- year-old girl who admitted to our hospital with fever, arthralgia and rash and diagnosed with sacroiliitis due to FMF and SPD.

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1. Yalçmkaya F, Tekin M, Turner N,Ozkaya N.Protracted Arthritis Of Familial Mediterranean Fever. Br J Rheumatol 1997; 36:1228- 1230.
2. Eifan AO, Ozdemir C, Aydogan M, Gocmen I, Bahçeciler NN, Bar- Ian IB. Incomplete attack and protracted sacroiliitis: an unusual manifestation of FMF in a child. Eur J Pediatr. 2007 Apr;166(4):383- 384.
3. KocakM, Birol A, Erkek E, Bozdoğan O, Atasoy P. Juvenile Subcorneal Pustular Dermatosis: A Case Report. Pediatric Dermatology 2003;20:57-59
4. Brodey P, Wolf SM. Radiographic Changes İn The Sacroiliac Joints İn Familial Mediterranean Fever. Radiology 1975; 114: 331-336.
5. Kast Kastner DL.Intermittent And Periodic Arthritis Syndromes. In: Koopman WJ (Ed) Arthritis And Allied Conditions. A Textbook Of Rheumatology, 14th Edn. Lippincott Williamsand Wilkins, Philadelphia, PA, 2001, Pp 1400-1143.
6. Langevitz P, Livneh A, Zemer D, Shemer J, Pras M.Seronegative Spondyloarthropathy İn Familial Mediterraneanfever. Semin Arthritis Rheum 1997; 27(2):67-726.
7. Beşbaş N,Özdemir S, Satçi I, Bakkaloğlu A, Özen S, Saatçi Ü.Sacroileitis İn Familial Mediterranean Fever. The Turkish Journal Of Pediatrics 1999; 41:387-390.
8. Connemann BJ,Steinhoff J, Benstein R, Sack K.Sakroiliitis Bei Familiarem Mittelmeerfieber. Dtsch Med Wschr 1991;116:1783-1787.
9. Lehman TJ,Hanson V, Kornreich H,Peters RS, Schwabe AD. HLA-B 27-Negatif Sacroileitis: Amanifestation Of Familial Mediterranean Fever İn Childhood. Pediatrics 1978; 61: 423-442.
10. Majeed H.A, Quabazard Z, Hıyazı Z, Farwana S, Harshanı F. The Cutaneous Manifestations in Children with Familial Mediterranean Fever . Quarterly Journal Of Medicine, New Series 1990;75, No.278;607-616.
11. Daisuke Tsuruta,, Akiko Matsumura-Oura And Masamitsu Ishii. Subcorneal Pustular Dermatosis And Sjögren's Syndrome. International Journal Of Dermatology 2005; 44:955-957
12. Modschiedler K, Weller M, Wo Rl P, Driesch P. Dapsone And Colchicine inhibit Adhesion Of Neutrophilic Granulocytes To Epidermal Sections. Arch Dermatol Res 2000 ; 292:32-36.