Evaluation of Pathergy Test Positivity in Familial Mediterranean Fever Patients and Comparison of Clinical Manifestations of FMF with Behçet's Disease

Overview

Journal Clin Rheumatol

Publisher Springer

Specialty Rheumatology

Date 2009 Jul 30

PMID 19639361

Citations 3

Authors

Fatma Aydin

Tekin Akpolat

Nilgun Senturk

Hasan Bagci

Ahmet Yasar Turanli

Affiliations

Soon will be listed here.

Abstract

Familial Mediterranean fever (FMF) shares a number of features with Behçet's disease (BD), such as their common ethnic origin, etiopathogenetic mechanisms, symptoms, and treatment. Pathergy reaction is accepted as a major criterion in BD. We aimed to determine the frequency of pathergy positivity in FMF patients and compared clinical features between FMF and BD. Pathergy test was performed in patients with FMF, BD, and healthy controls. Diagnostic criteria for FMF and BD were screened in both groups. None of the FMF patients or healthy controls yielded positive pathergy test. Pathergy test was positive in 13 out of 31 (41.9%) of the patients with BD. None of the FMF patients fulfilled the International Study Group criteria for BD. None of the BD patients fulfilled the Livneh diagnostic criteria for FMF. BD and FMF are associated with neutrophilic dermatoses and neutrophil hyper-reactivity. Although pathergy test and erysipelas-like erythema share some histological findings, pathergy test was negative among FMF patients.

Citing Articles

Diagnostic Sensitivity of Different Applications of Pathergy Test for Behçet's Disease.

Yildizhan I, Boyvat A Arch Rheumatol. 2020; 35(1):29-34.

PMID: 32637917 PMC: 7322300. DOI: 10.5606/ArchRheumatol.2020.7380.

Familial mediterranean fever in an Iranian patient with behcet disease.

Mobini M Caspian J Intern Med. 2014; 2(4):344-6.

PMID: 24551444 PMC: 3895835.

Evaluation of nailfold capillaries in familial Mediterranean fever patients.

Aytekin S, Aydin F, Akpolat T, Senturk N, Turanli A Clin Rheumatol. 2010; 30(2):255-7.

PMID: 21053037 DOI: 10.1007/s10067-010-1609-x.

References

Mege J, Dilsen N, Sanguedolce V, Gul A, Bongrand P, Roux H . Overproduction of monocyte derived tumor necrosis factor alpha, interleukin (IL) 6, IL-8 and increased neutrophil superoxide generation in Behçet's disease. A comparative study with familial Mediterranean fever and healthy subjects. J Rheumatol. 1993; 20(9):1544-9. View

Rabinovich E, Shinar Y, Leiba M, Ehrenfeld M, Langevitz P, Livneh A . Common FMF alleles may predispose to development of Behcet's disease with increased risk for venous thrombosis. Scand J Rheumatol. 2007; 36(1):48-52. DOI: 10.1080/03009740600759639. View

Direskeneli H, Ozdogan H, Korkmaz C, Akoglu T, Yazici H . Serum soluble intercellular adhesion molecule 1 and interleukin 8 levels in familial Mediterranean fever. J Rheumatol. 1999; 26(9):1983-6. View

Chang H, Cheon K . The clinical significance of a pathergy reaction in patients with Behcet's disease. J Korean Med Sci. 2002; 17(3):371-4. PMC: 3054891. DOI: 10.3346/jkms.2002.17.3.371. View

Hatemi I, Hatemi G, Celik A, Melikoglu M, Arzuhal N, Mat C . Frequency of pathergy phenomenon and other features of Behçet's syndrome among patients with inflammatory bowel disease. Clin Exp Rheumatol. 2008; 26(4 Suppl 50):S91-5. View

Bi X, Gu J, Yan M, Gao C . A case of Sweet's syndrome with slack skin and pathergy phenomenon. Int J Dermatol. 2008; 47(8):842-4. DOI: 10.1111/j.1365-4632.2008.03689.x. View

Lindberg-Larsen R, Fogh K . Traumatic pyoderma gangrenosum of the face: pathergy development after bike accident. Dermatology. 2008; 218(3):272-4. DOI: 10.1159/000182266. View

Ozdogan H, Arisoy N, Kasapcapur O, Sever L, Caliskan S, Tuzuner N . Vasculitis in familial Mediterranean fever. J Rheumatol. 1997; 24(2):323-7. View

Miyoshi T, Yamashita K, Ohno T, Izumi T, Takaori-Kondo A, Sasada M . Familial Mediterranean fever gene as a possible modifier of Sweet syndrome with chronic myelogenous leukemia. Acta Haematol. 2008; 120(1):57-62. DOI: 10.1159/000158578. View

10.

Yurdakul S, Yazici H . Behçet's syndrome. Best Pract Res Clin Rheumatol. 2008; 22(5):793-809. DOI: 10.1016/j.berh.2008.08.005. View

11.

Ghate J, Jorizzo J . Behçet's disease and complex aphthosis. J Am Acad Dermatol. 1999; 40(1):1-18; quiz 19-20. DOI: 10.1016/s0190-9622(99)70523-2. View

12.

Uzun O, Akpolat T, Erkan L . Pulmonary vasculitis in behcet disease: a cumulative analysis. Chest. 2005; 127(6):2243-53. DOI: 10.1378/chest.127.6.2243. View

13.

Jorizzo J, Solomon A, CAVALLO T . Behçet's syndrome. Immunopathologic and histopathologic assessment of pathergy lesions is useful in diagnosis and follow-up. Arch Pathol Lab Med. 1985; 109(8):747-51. View

14.

Gul A, Esin S, Dilsen N, Konice M, Wigzell H, Biberfeld P . Immunohistology of skin pathergy reaction in Behçet's disease. Br J Dermatol. 1995; 132(6):901-7. DOI: 10.1111/j.1365-2133.1995.tb16946.x. View

15.

Renda M, Civitavecchia G, Fabiano C, Sammarco P, Cottone M . Familial mediterranean fever gene (MEVF) mutations in Crohn's disease in a Mediterranean area. Inflamm Bowel Dis. 2008; 14(9):1313. DOI: 10.1002/ibd.20424. View

16.

Barzilai A, Langevitz P, Goldberg I, Kopolovic J, Livneh A, Pras M . Erysipelas-like erythema of familial Mediterranean fever: clinicopathologic correlation. J Am Acad Dermatol. 2000; 42(5 Pt 1):791-5. DOI: 10.1067/mjd.2000.103048. View

17.

. Familial Mediterranean fever (FMF) in Turkey: results of a nationwide multicenter study. Medicine (Baltimore). 2005; 84(1):1-11. DOI: 10.1097/01.md.0000152370.84628.0c. View

18.

Inaloz H, Evereklioglu C, Unal B, Kirtak N, Eralp A, Inaloz S . The significance of immunohistochemistry in the skin pathergy reaction of patients with Behçet's syndrome. J Eur Acad Dermatol Venereol. 2003; 18(1):56-61. DOI: 10.1111/j.1468-3083.2004.00547.x. View

19.

Yigit S, Bagci H, Ozkaya O, Ozdamar K, Cengiz K, Akpolat T . MEFV mutations in patients with familial Mediterranean fever in the Black Sea region of Turkey: Samsun experience [corrected]. J Rheumatol. 2007; 35(1):106-13. View

20.

Ozen S, Ben-Chetrit E, Bakkaloglu A, Gur H, Tinaztepe K, Calguneri M . Polyarteritis nodosa in patients with Familial Mediterranean Fever (FMF): a concomitant disease or a feature of FMF?. Semin Arthritis Rheum. 2001; 30(4):281-7. DOI: 10.1053/sarh.2001.19958. View