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Focal Eosinophilic Myositis Associated with Behçet's Disease

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Focal Eosinophilic Myositis Associated with Behçet's Disease pISSN: 2093-940X, eISSN: 2233-4718 Journal of Rheumatic Diseases Vol. 24, No. 5, October, 2017 https://doi.org/10.4078/jrd.2017.24.5.303 Case Report Focal Eosinophilic Myositis Associated with Behçet's Disease Jung Su Eun1, Jong Wan Kang1, Jin Young Kang2, Na Ri Kim1, Sang Jin Lee1, Young Mo Kang1, Man Hoon Han3, Eon Jeong Nam1 1Division of Rheumatology, Department of Internal Medicine, Kyungpook National University School of Medicine, Daegu, 2Division of Rheumatology, Department of Internal Medicine, Changwon Fatima Hospital, Changwon, 3Department of Pathology, Kyungpook National University School of Medicine, Daegu, Korea Behçet’s disease (BD) is a systemic vasculitis commonly accompanied by recurrent mucosal ulceration and other systemic man- ifestations, but rarely by myositis. Focal eosinophilic myositis is the most limited idiopathic eosinophilic myopathy charac- terized by peripheral blood eosinophilia and/or eosinophilic muscle infiltration. Clinical manifestations include myalgia, mus- cle weakness, and cutaneous lesions, such as subcutaneous induration and erythema. Given that BD can mimic deep vein thrombosis or pseudotumor, muscle biopsy should be performed to enhance the accuracy of diagnosis. Microscopic examina- tion reveals extensive infiltration of eosinophils and mononuclear cells into muscle, myofiber necrosis, and regeneration. To the best of our knowledge, there have not been any published reports on MEDLINE regarding focal eosinophilic myositis asso- ciated with BD. Here, we presented a case of focal eosinophilic myositis associated with intestinal BD in a 23-year-old man who suffered from a large ulcer in the terminal ileum. (J Rheum Dis 2017;24:303-308) Key Words. Behçet’s disease, Idiopathic eosinophilic myopathy, Focal eosinophilic myositis INTRODUCTION ositis [1,6]. Eosinophilic infiltration into skeletal muscle, although Behçet’s disease (BD) is a systemic vasculitis, charac- rare, has been described in a diverse group of conditions, terized by vascular injury, hyperfunction of neutrophils, including drugs such as L-tryptophan, D-penicillamine and autoimmune responses and clinically often presented and ethanol, parasitic infection, autoimmune diseases with mucous membrane ulceration, skin lesions and ocu- such as rheumatoid arthritis, myasthenia gravis and eosino- lar symptoms [1,2]. Gastrointestinal (GI) involvement in philic granulomatosis with polyangiitis, eosinophilia-myal- BD is particularly important as it is one of serious and dif- gia syndrome, and idiopathic eosinophilic myopathy ficult to treat; therefore, BD is designated ‘‘intestinal [7,8]. Although pathogenesis of eosinophilic myopathies BD,’’ if a typical oval-shaped large ulcer in the terminal is incompletely understood, it is suggested that inter- ileum or ulcerations in the GI tract are objectively docu- leukin-5 is stimulated by a precipitating factor, and the in- mented [2,3]. Musculoskeletal involvement is one of the flammatory mediators by eosinophil including cationic most frequent findings in BD, of which arthritis and ar- protein, eosinophilic major basic protein, and enzymes, thralgia are the most common findings [1,4]. Myositis is increase in the target tissues and blood [8]. Idiopathic eo- rare manifestation in BD and is usually mild, short-last- sinophilic myopathy is a rare, clinically and pathologically ing, and more localized than generalized form [5]. Histologic heterogeneous disease, characterized by the presence of examination reveals a predominant neutrophil infiltra- peripheral and/or muscle eosinophilia [8]. Clinical mani- tion associated with focal necrosis and perivasculitis, sug- festations include myalgia, muscle weakness, edematous gesting vasculitis participates in the pathogenesis of my- changes of extremities, arthralgia/arthritis, and skin Received：March 21, 2017, Revised：April 12, 2017, Accepted：April 19, 2017 Corresponding to：Eon Jeong Nam, Division of Rheumatology, Department of Internal Medicine, Kyungpook National University Hospital, 130 Dongdeok-ro, Jung-gu, Daegu 41944, Korea. E-mail：[email protected] Copyright ⓒ 2017 by The Korean College of Rheumatology. All rights reserved. This is a Open Access article, which permits unrestricted non-commerical use, distribution, and reproduction in any medium, provided the original work is properly cited. 303 Jung Su Eun et al. lesions. Cutaneous manifestations include deep subcuta- covered by normal skin at the back of right upper arm and neous induration, erythema, urticarial or angioedema- several subcutaneous induration with tenderness of the tous plaques, and erythematous papular lesions, in which left flank and the right lower leg. In manual muscle test, deep subcutaneous induration is the most common find- there was no evidence of muscle weakness of both upper ing [9,10]. Biopsy of cutaneous lesions demonstrates and lower extremities. Pathergy test was positive. scanty-to-moderate perivascular mononuclear infiltrates Laboratory examination revealed a white blood cell with a considerable variability in the number of admixed count of 9,890/μL (eosinophil count 380/μL, 3.8%), er- eosinophils, ranging from lymphocytic vasculitis devoid ythrocyte sedimentation rate of 40 mm/h (normal, 0∼ of eosinophils to eosinophilic cellulitis-like lesions [9]. 10), C-reactive protein of 2.13 mg/dL (0∼0.5), creatine Most, if not all, of the patients represent peripheral or tis- phosphokinase of 33 μL (39∼308), lactate dehydrogen- sue eosinophilia, and elevated muscle enzymes [10]. ase of 392 μL (0∼250), myoglobin of 18 ng/mL (14∼ Although connective tissue disorders may be related 106), and aldolase of 15.4 μL (0∼7.6). Results of im- with eosinophilic myopathy [8] and the localized form of munological analyses were as follows: immunoglobulin myopathy can be presented as the musculoskeletal mani- (Ig) G 1,643 mg/dL (700∼1,600), IgA 237 mg/dL (70∼ festation of BD [4,5], there are no published case reports 400), IgM 154 mg/dL (94∼230), C3 132.3 mg/dL (90∼ that the localized form of myopathy in patients with BD 180), C4 34.9 mg/dL (10∼40), antinuclear antibody neg- was identified as focal eosinophilic myositis when sear- ative, rheumatoid factor negative, and anti-neutrophil cy- ched on MEDLINE. Herein, we present a case of focal eo- toplasmic antibody negative. sinophilic myositis associated with intestinal BD in a Chest X-ray, electrocardiogram, and echocardiogram 23-year-old man who suffered from a large ulcer in the findings were all nonspecific. The patient complained of terminal ileum. persistent abdominal pain and underwent abdominal computed tomography (CT) and colonoscopy. Abdominal CASE REPORT CT showed a wall thickening at the terminal ileum (Figure 1A), and colonoscopy revealed a huge, round A 23-year old man who had a history of recurrent oral ul- well-demarcated ulcer with central yellowish exudate at cerations and pseudofolliculitis was admitted to our hos- the terminal ileum (Figure 1B). The mucosal biopsies of pital with abdominal pain and a mass in the right upper intestinal lesions showed chronic inflammation without arm. Six months ago, he began to have recurrent right granuloma. Magnetic resonance image (MRI) study of the lower abdominal pain, several subcutaneous nodules, right upper arm demonstrated a focal mass lesion meas- and, at the same time, a persistent painful firm mass ap- uring 3.0 cm (length)×2.0 cm (width) in the right triceps peared on the right upper arm. He had no past history of muscle with a heterogeneous hyper-intense signal on drug medication or allergic diseases. The abdominal ex- T2-weighted image (Figure 2A). On a gadolinium en- amination revealed tenderness in right lower quadrant. hancement T1-weighted image of MRI, the lesions ap- There were about a 3 cm-sized firm, nonmovable mass peared to be a well-defined rim of contrast enhancement Figure 1. (A) Abdominal con- trast-enhanced computed to- mography shows a wall thick- ening and mucosal enhancement at the terminal ileum (arrows), suggesting terminal ileitis. (B) Colonoscopy reveals a large well-demarcated ulcerative le- sion with central yellowish exu- date at the terminal ileum. 304 J Rheum Dis Vol. 24, No. 5, October, 2017 Focal Eosinophilic Myositis Associated with Behçet's Disease Figure 2. Magnetic resonance imaging of right upper arm. (A) An axial T2-weighted image de- monstrates diffuse and irregular hyperintensity signal around a focal mass lesion in triceps muscle. (B) An axial gadolini- um-enhanced T1-weighted im- age shows a focal mass lesion consisting of a well-defined rim of contrast enhancement (arrows) and a hypointense central area suspected of necrosis (arrow- head). Figure 3. Histopathologic analysis of muscle. (A) Normal muscle structure (arrowheads) is observed sparsely, and the remainder is replaced by inflammatory cells and necrotic tissues (arrows) (H&E stain, ×40). (B) At higher magnification of the necrotic tissue (A, white arrow), the necrosis is surrounded by abundant inflammatory cells and eosinophils (H&E stain, ×400). (C, D) Infiltrations of eosinophils (C) and CD3+ T lymphocytes (D) are prominent in tissue (C: H&E stain, ×400; D: CD3 immunohistochemical stain, ×400). and a hypointense central area (Figure 2B). Muscle biop- atrophic and were replaced by inflammatory cell infiltra- sy, performed to determine the exact pathologic findings tion with predominant eosinophils, and focal central ne- of triceps muscle, showed that myofibers became severe crosis without perivasculitis, which were consistent with www.jrd.or.kr 305 Jung Su Eun et
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