Diffuse Skin Tightness in Familial Mediterranean Fever: A Case Report and Review of Literature
Abstract
Introduction: Familial Mediterranean Fever (FMF) is an autosomal recessive inherited disorder that has skin presentations like vasculitis and paniculitis. There has not been shown any association between systemic sclerosis and FMF in studies. Hence, we report a case of FMF with diffuse skin tightness. Case Presentation: An 18-year-old girl known as a case of FMF for 3 years presents to our department with tight skin since childhood. The stiffness of skin appeared when she was about 7 years old. In physical examination, tight skin with general induration in all surfaces of skin, sclerodactyly and beaked nose microstomia are noticed. The findings of all hematological, biochemical and pathological studies were normal. Conclusion: Finally, it may be concluded that FMF should be considered as a differential diagnosis in patients attending with skin tightness and the possible etiology is cytokines. [GMJ.2015;4(4):169-72]References
Akar S, Soysal O, Balci A, Solmaz D, Gerdan V, Onen F, et al. High prevalence of spondyloarthritis and ankylosing spondylitis among familial Mediterranean fever patients and their first-degree relatives: further evidence for the connection. Arthritis Res Ther. 2013;15(1):R21.
Petrushkin H, Stanford M, Fortune F, Jawad AS. Clinical Review: Familial Mediterranean Fever-An Overview of Pathogenesis, Symptoms, Ocular Manifestations, and Treatment. Ocul Immunol Inflamm. 2015;24:1-9.
Mobini M. Familial mediterranean fever in an Iranian patient with behcet disease. Caspian J Intern Med. 2011;2(4):344-6.
Deniz R, Ozen G, Yilmaz-Oner S, Alibaz-Oner F, Erzik C, Aydin SZ et al. Familial Mediterranean fever gene (MEFV) mutations and disease severity in systemic lupus erythematosus (SLE): implications for the role of the E148Q MEFV allele in inflammation. Lupus. 2015;24(7):705-11.
Ghosh K. Familial Mediterranean fever: An unusual disease enlightening the inflammation biology. Indian J Hum Genet. 2014;20(1):1-3.
Medlej-Hashim M, Nehme N, Chouery E, Jalkh N, Megarbane A. Novel MEFV transcripts in Familial Mediterranean fever patients and controls. BMC Med Genet. 2010;11:87.
Akman A, Cakcak DS, Coban E, Ozbudak HI, Ciftcioglu MA, Alpsoy E, et al. Recurrent bullous lesions associated with familial Mediterranean fever: a case report. Clin Exp Dermatol. 2009; 34(2):216-8.
Apostolidou E, Kambas K, Chrysanthopoulou A, Kourtzelis I, Speletas M, Ritis K et al. Genetic analysis of C5a receptors in neutrophils from patients with familial Mediterranean fever. Mol Biol Rep. 2012;39(5):5503-10.
Bafounta ML, Doumat-Batch F, Vasseur E, Staroz F, Clerici T, Saiag P. Unusual cutaneous lesions of familial Mediterranean fever.Ann Dermatol Venereol. 2004; 131(2):183-6.
Savic S, Dickie LJ, Battellino M, McDermott MF. Familial Mediterranean fever and related periodic fever syndromes/autoinflammatory diseases. Curr Opin Rheumatol. 2012;24(1):103-12.
Marcuzzi A, Piscianz E, Kleiner G. Clinical genetic testing of periodic fever syndromes. Biomed Res Int. 2013; 2013:501305.
Guz G, Kanbay M, Ozturk MA. Current perspectives on familial Mediterranean fever.Curr Opin Infect Dis. 2009;22(3):309-15.
Viswanath V, Phiske MM, Gopalani VV. Systemic sclerosis: current concepts in pathogenesis and therapeutic aspects of dermatological manifestations. Indian J Dermatol. 2013;58(4):255-68.
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