American Journal of Medical Case Reports
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American Journal of Medical Case Reports. 2019, 7(8), 170-172
DOI: 10.12691/ajmcr-7-8-5
Open AccessCase Report

Juvenile Dermatomyositis Associated to Familial Mediterranean Fever

Salem Bouomrani1, 2, , Rim Mesfar1, 2, Moez BenAyed2, 3, Mouna Guermazi1, 2, Hassène Baïli1 and Souad Yahyaoui1

1Department of Internal Medicine, Military Hospital of Gabes, Gabes 6000, Tunisia

2Sfax Faculty of Medicine, University of Sfax, Sfax 3029, Tunisia

3Department of Orthopedics, Regional Hospital of Gabes, Gabes 6000, Tunisia

Pub. Date: July 11, 2019

Cite this paper:
Salem Bouomrani, Rim Mesfar, Moez BenAyed, Mouna Guermazi, Hassène Baïli and Souad Yahyaoui. Juvenile Dermatomyositis Associated to Familial Mediterranean Fever. American Journal of Medical Case Reports. 2019; 7(8):170-172. doi: 10.12691/ajmcr-7-8-5


Familial Mediterranean Fever (FMF) is a rare hereditary auto-inflammatory disease that can be exceptionally associated with many other dys-immune disorders; the most reported associations were with systemic vasculitis, spondyloarthropathies, inflammatory bowel diseases, systemic lupus erythematous, multiple sclerosis, and juvenile chronic arthritis. The association of FMF with primary inflammatory myopathy remains exceptional and unusual; it has only been noted once before with adult polymyositis. We report an original observation of FMF associated with juvenile dermatomyositis in an eight-year-old boy, which, to our knowledge, has not been reported previously.

familial mediterranean fever juvenile dermatomyositis dermatomyositis

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[1]  Ozdogan H, Ugurlu S. Familial Mediterranean Fever. Presse Med. 2019 Jan 24. pii: S0755-4982(18)30486-X.
[2]  Georgin-Lavialle S, Fayand A, Rodrigues F, Bachmeyer C, Savey L, Grateau G. Autoinflammatory diseases: State of the art. Presse Med. 2019 Jan 24. pii: S0755-4982(18)30485-8.
[3]  Sedivá A, Horváth R, Maňásek V, Gregorová A, Plevová P, Horáčková M, et al. Cluster of patients with Familial Mediterranean fever and heterozygous carriers of mutations in MEFV gene in the Czech Republic. Clin Genet. 2014; 86(6): 564-9.
[4]  Özçakar ZB, Çakar N, Uncu N, Çelikel BA, Yalçinkaya F. Familial Mediterranean fever-associated diseases in children. QJM. 2017; 110(5): 287-290.
[5]  Bouomrani S, Guermazi M. Multiple Sclerosis and Familial Mediterranean Fever: Myth or Reality?. Ann Mult Scler Res. 2019; 1(1): 1003.
[6]  Eguchi M, Miyashita T, Shirouzu H, Sato S, Izumi Y, Takeoka A, et al. Coexistence of polymyositis and familial Mediterranean fever. Mod Rheumatol. 2013; 23(2): 374-8.
[7]  Hofer MF, Rivier G, Guerne PA, Girardin E. La dermatomyosite juvénile: une maladie différente de la forme adulte? Rev Med Suisse. 2001; 3: 21264.
[8]  Pelkonen PM, Jalanko HJ, Lantto RK, Mäkelä AL, Pietikäinen MA, Savolainen HA, et al. Incidence of systemic connective tissue diseases in children: a nationwide prospective study in Finland. J Rheumatol. 1994; 21(11): 2143-6.
[9]  Bader-Meunier B, Gitiaux C, Belot A, Brochard K, Mouy R, Ponce D, et al. French expert opinion for the management of juvenile dermatomyositis. Arch Pediatr. 2019; 26(2): 120-125.
[10]  McCann LJ, Pain CE. A Practical Approach to Juvenile Dermatomyositis and Juvenile Scleroderma. Indian J Pediatr. 2016; 83(2): 163-71.
[11]  Estublier C, Stankovic Stojanovic K, Bergerot JF, Broussolle C, Sève P. Myositis in a patient with familial Mediterranean fever and spondyloarthritis successfully treated with anakinra. Joint Bone Spine. 2013; 80(6): 645-9.
[12]  Gdynia HJ, Sperfeld AD, Haerter G. Histologic signs of inflammatory myopathy in familial Mediterranean fever. J Clin Rheumatol. 2006; 12(5): 265-6.
[13]  Akl K, Rawashdeh MO. Isolated myositis as a sole presentation of familial Mediterranean fever. Rheumatol Int. 2012; 32(10): 3321-2.
[14]  Tattevin P, Arondelle C, Margent P, Broucker De T. Neuromuscular complications of long-term treatment of inflammatory diseases. 3 cases. Ann Med Interne (Paris). 1999; 150(8): 594-7.