Dear Label_Name,

Greetings! I hope you are well.

I am writing on behalf of an international publishing house, Science and Education Publishing, USA (http://www.sciepub.com).

Science and Education Publishing (SciEP) is a multidisciplinary open access publisher of Journals covering the fields of Science, Technology and Medicine. All SciEP publications are subject to high-quality peer review, editorial and production processes and are freely available online.

Welcome to the April, 2013 edition of SciEP newsletter, providing monthly summaries of articles on science & education related topics of interest to scientist and professionals. The mission of our journals is to provide an open access forum for publishing new findings on issues pertinent to scientific and academic field. We also invite you to submit original research papers, review articles, short reports, or case studies are considered for publication in our Multidisciplinary Journals.

American Journal of Public Health Research
DOI: 10.12691/ajphr-1-1-3

The Implementation of a Tele-Homecare System with Service Oriented Architecture and HL7 Message Transmission Standard
Ching-Sung Wang

American Journal of Medical and Biological Research
DOI: 10.12691/ajmbr-1-1-2

The Influence of Ethanol on Pyruvate Kinases Activity in Vivo, in Vitro, in Silico
Lelevich Sergey Vladimirovich, Khrustalev Vladislav Victorovich, Barkovsky Eugene Victorovich, Shedogubova Tatyana Aleksandrovna

American Journal of Medical Sciences and Medicine
DOI: 10.12691/ajmsm-1-1-1

Clinical and Histological Features in Wegener Granulomatosis
Li Cavoli Gioacchino, Passantino Rita, Bono Luisa, Tortorici Calogera, Ferrantelli Angelo, Giammarresi Carlo, Rotolo Ugo

American Journal of Infectious Diseases and Microbiology
DOI: 10.12691/ajidm-1-1-2

The Curse of Lymphatic Filariasis: Would the Continual Use of Diethylcarbamazine Eliminate this Scourge in Papua New Guinea?
Anne Lanham, Lillian Mwanri

We have released issues of some journals on February 2013.
American Journal of Public Health Research
American Journal of Medical Sciences and Medicine
American Journal of Medical and Biological Research
American Journal of Pharmacological Sciences
American Journal of Clinical Medicine Research
American Journal of Nursing Research
American Journal of Cancer Prevention
American Journal of Medicine Studies
American Journal of Infectious Diseases and Microbiology
American Journal of Cardiovascular Disease Research
American Journal of Biomedical Research
American Journal of Mechanical Engineering
American Journal of Microbiological Research
Journal of Preventive Medicine
American Journal of Food and Nutrition
American Journal of Hypertension Research
American Journal of Sports Science and Medicine
Journal of Cancer Research and Treatment
American Journal of Zoological Research
Journal of Biomedical Engineering and Technology
American Journal of Microbiological Research
Biomedical Science and Engineering

We are pleased to announce that submissions are now being accepted for peer review for all the journals across all our subject areas in SciEP.

Please submit your manuscript via our online submission system:
http://www.sciepub.com/author/signin

To unsubscribe from our mailing list, please click on the following link: unsubscribe

I am looking forward to hearing from you.

Kind regards,

David Rain
Email:join@sciepub.com
Science and Education Publishing (http://www.sciepub.com)

Juvenile Dermatomyositis Associated to Familial Mediterranean Fever

Salem Bouomrani*, Rim Mesfar, Moez BenAyed, Mouna Guermazi, Hassène Baïli, Souad Yahyaoui

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

Abstract: 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.

Keywords: familial mediterranean fever, juvenile dermatomyositis, dermatomyositis

Received May 10, 2019; Revised June 19, 2019; Accepted July 11, 2019

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

1. Introduction

Familial Mediterranean Fever (FMF) is a rare hereditary auto-inflammatory disease [1,2], which is particularly common among Sephardic Jews, Turkish, Armenian, and Arab, where the prevalence may be in the order of 1-2/1000 [1]. It is currently recognized as a ubiquitous disease, although in most cases the notion of an ancestor from the Middle East or the Mediterranean region is found [1,3]. This disease is genetically determined, caused by a mutation in the MEFV gene on chromosome 16 (16p13.3), and its transmission is predominantly autosomal recessive [1,2,3].

The association of FMF with other dys-immune disorders remains exceptional; the most reported associations were with systemic vasculitis, spondyloarthropathies, inflammatory bowel diseases, systemic lupus erythematous, multiple sclerosis, and juvenile chronic arthritis [1,4,5].

The association of FMF with primary inflammatory myopathy remains exceptional and unusual; it has only been noted once before with polymyositis [6].

We report an original case of FMF associated with juvenile dermatomyositis (JDM), which, to the best of our knowledge, has not been reported previously.

2. Case Report

An eight-year-old boy whith known FMF presented to our department with a feverish eruption of the face and hands.

The diagnosis of FMF was made at the age of 6 in front of recurrent abdominal pain with bilateral pleurisy, and confirmed by the presence of the M694V homozygous mutation of the MEFV gene. He was treated with colchicine with a good response.

The somatic examination found a febrile child at 38°C, a heliotrope erythrœdema of the face (Figure 1), Gottron's papules of the extension faces of the proximal and distal inter-phalangeal and metacarpo-phalangeal joints of both hands (Figure 2), and provoked diffuse myalgia without muscle deficit.

Biology showed a marked inflammatory syndrome with erythrocyte sedimentation rate at 68 mm/H1 and a C-reactive protein at 42 mg/l, and rhabdomyolysis with creatinine kinase at 320 IU/l and lactic dehydrogenase at 480 IU/l. Other basic bioassays were within normal limits: total blood count, creatinine, serum calcium, fast glycaemia, transaminases, lipid parameters, and thyroid hormones. Standard X-rays of the chest and hands were without abnormalities.

Antinuclear antibodies were positive at 1/320 with positive anti-Mi2 antibodies. The electromyogram revealed a myogenic syndrome of the four limbs without associated neurogenic signs. Skin-muscle biopsy confirmed the diagnosis of acute dermatomyositis and did not show signs of colchicine-induced myopathy. No systemic visceral involvement was found, and investigations for underlying cancer were negative.

Under systemic corticosteroids at a dose of 1mg/kg/day and hydroxychloroquine at a dose of 200 mg/day, the evolution was rapidly favorable with complete remission.

Figure 1. Heliotrope erythrœdema of the face

Figure 2. Gottron's papules on both hands

3. Discussion

Juvenile dermatomyositis (JDM) is a primary inflammatory myopathy of unknown etiology occurring, by definition, in children under 16 years of age [7,8,9]. Its incidence is estimated at 0.3-0.4/100,000 children, and is the most common inflammatory myopathy of the child with a maximum incidence around the age of seven [7,8,9]. It can rarely associate with other autoimmune diseases, particularly systemic lupus erythematous, systemic sclerosis, and primary Sjögren's syndrome [10]. His association with the FMF has not been reported before. Indeed, in the Özçakar ZB et al series of 600 children with FMF, of whom 77 had concomitant disease, no association with JDM was noted [4].

This association represents a real diagnostic challenge for clinicians, especially since localized or diffuse myositis may be a clinical manifestation of FMF [11,12], and may even be the only manifestation revealing it [13]. Similarly, colchicine, a basic drug for the treatment of FMF can induce an iatrogenic neuromuscular pathology that can simulate polymyositis (polumyositis-like syndrome) [14].

The association of FMF with an authentic primary inflammatory myopathy has been reported only once in the world literature by Eguchi M et al [6]. It was the case of a 37-year-old Japanese woman with FMF associated to a polymyositis. The exact mechanism of this association is not yet well understood, but this association comforts once again the immune dysfunction induced by FMF [6].

4. Conclusion

The association of FMF with primary inflammatory myositis remains exceptional and unusual. It deserves to be known because it can represent real diagnostic and therapeutic challenges.

Our observation is, to the best of our knowledge, the first reporting the association of FMF to dermatomyositis. It is also distinguished by its juvenile character.

Conflicts of Interest

None.

References

[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.