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. 2017 Oct 27;5(1):e410.
doi: 10.1212/NXI.0000000000000410. eCollection 2018 Jan.

Brachio-cervical inflammatory myopathy with associated scleroderma phenotype and lupus serology

Affiliations

Brachio-cervical inflammatory myopathy with associated scleroderma phenotype and lupus serology

Andrew F Gao et al. Neurol Neuroimmunol Neuroinflamm. .
No abstract available

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Figures

Figure
Figure. Muscle biopsy of the left biceps showing the characteristic pathologic findings in BCIM
(A.a, B) Cryostat sections stained with hematoxylin phloxine saffron show widespread muscle fiber atrophy without perifascicular distribution, endomysial fibrosis, and numerous necrotic and regenerating fibers. (A.b) Alkaline phosphatase staining of the perimysium. (C) Multiple inflammatory foci, with a perivascular and perimysial (D) and endomysial distribution (E). Germinal centers with polarization were occasionally seen (F), which stained for CD10 and bcl-6, but not bcl-2, ruling out follicular lymphoma (not shown). Immunohistochemistry showed that inflammatory infiltrates were composed of CD20+ B cells (G) along with CD4+ and CD8+ T cells (not shown). Multiple CD20+ B-cell foci were appreciated (H). Membrane attack complex (MAC) deposition was widespread in the endomysium, with several examples of MAC deposition around multiple fibers, confirming its endomysial location (I, green arrows).

References

    1. Pestronk A, Kos K, Lopate G, Al-Lozi MT. Brachio-cervical inflammatory myopathies: clinical, immune, and myopathologic features. Arthritis Rheum 2006;54:1687–1696. - PubMed
    1. Van der Kooi A, Jaeger B, Pinto Y, Aronica E, de Visser M. Two women with brachio-cervical inflammatory myopathy (BCIM) and fatal cardiomyopathy. Neuromuscul Disord 2015;25:S308. - PubMed
    1. Neutel D, Barroso C. Brachio-cervical inflammatory myopathy—a case report. Neuromuscul Disord 2011;21:733–734.
    1. Khadilkar SV, Gupta N, Yadav RS. Cervicobrachial polymyositis. J Clin Neuromuscul Dis 2014;16:59–68. - PubMed
    1. Garcin B, Lenglet T, Dubourg O, Mesnage V, Levy R. Dropped head syndrome as a presenting sign of scleromyositis. J Neurol Sci 2010;292:101–103. - PubMed

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