Focal Eosinophilic Myositis Associated with Behçet's Disease
- Affiliations
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- 1Division of Rheumatology, Department of Internal Medicine, Kyungpook National University School of Medicine, Daegu, Korea. ejnam@knu.ac.kr
- 2Division of Rheumatology, Department of Internal Medicine, Changwon Fatima Hospital, Changwon, Korea.
- 3Department of Pathology, Kyungpook National University School of Medicine, Daegu, Korea.
- KMID: 2394346
- DOI: http://doi.org/10.4078/jrd.2017.24.5.303
Abstract
- Behçet's disease (BD) is a systemic vasculitis commonly accompanied by recurrent mucosal ulceration and other systemic manifestations, but rarely by myositis. Focal eosinophilic myositis is the most limited idiopathic eosinophilic myopathy characterized by peripheral blood eosinophilia and/or eosinophilic muscle infiltration. Clinical manifestations include myalgia, muscle 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 examination 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 associated 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.
MeSH Terms
Figure
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Figure 1 (A) Abdominal contrast-enhanced computed tomography shows a wall thickening and mucosal enhancement at the terminal ileum (arrows), suggesting terminal ileitis. (B) Colonoscopy reveals a large well-demarcated ulcerative lesion with central yellowish exudate at the terminal ileum.
Figure 2 Magnetic resonance imaging of right upper arm. (A) An axial T2-weighted image demonstrates diffuse and irregular hyperintensity signal around a focal mass lesion in triceps muscle. (B) An axial gadolinium-enhanced T1-weighted image shows a focal mass lesion consisting of a well-defined rim of contrast enhancement (arrows) and a hypointense central area suspected of necrosis (arrowhead).
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).
Figure 4 Follow-up magnetic resonance imaging (MRI) of right upper arm. (A) On axial T2-weighted image, the signal intensity and extent of the involved muscle have been distinctly decreased compared to the initial MRI findings. (B) An axial gadolinium-enhanced T1-weighted image reveals reduced size of focal mass lesion (arrows) and disappearance of internal necrosis, which was evident in previous MRI.
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