eMedicine Specialties > Neurology > Neuromuscular Diseases

Dermatomyositis/Polymyositis: Multimedia

Author: Sushma Podila, MD, Resident Physician, Department of Neurology, Columbia Presbyterian Medical Center
Coauthor(s): Thomas H Brannagan III, MD, Associate Professor of Clinical Neurology and Director, Peripheral Neuropathy Center, Columbia University, College of Physicians and Surgeons; Co-Director, EMG Laboratory, New York-Presbyterian Hospital, Columbia Campus, New York
Contributor Information and Disclosures

Updated: Aug 28, 2009

Multimedia

Hematoxylin and eosin paraffin section shows poly...
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Media file 1: Hematoxylin and eosin paraffin section shows polymyositis. Longitudinal section shows a dense, chronic, endomysial inflammatory infiltrate. Image courtesy of Roberta J. Seidman, MD.
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Hematoxylin and eosin paraffin section shows poly...

Hematoxylin and eosin paraffin section shows polymyositis. Longitudinal section shows a dense, chronic, endomysial inflammatory infiltrate. Image courtesy of Roberta J. Seidman, MD.

Hematoxylin and eosin frozen section shows polymy...
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Media file 2: Hematoxylin and eosin frozen section shows polymyositis. Endomysial chronic inflammation is present among intact myofibers that are remarkable only for increased variability of fiber size. Image courtesy of Roberta J. Seidman, MD.
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Hematoxylin and eosin frozen section shows polymy...

Hematoxylin and eosin frozen section shows polymyositis. Endomysial chronic inflammation is present among intact myofibers that are remarkable only for increased variability of fiber size. Image courtesy of Roberta J. Seidman, MD.

Hematoxylin and eosin paraffin section shows poly...
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Media file 3: Hematoxylin and eosin paraffin section shows polymyositis. Patient had dense endomysial inflammation that contains an abundance of plasma cells, which can be observed in patients with chronic polymyositis. Two necrotic myofibers, characterized by dense eosinophilic staining, are observed. Focal fatty infiltration of the muscle is present in the lower left quadrant of the photomicrograph. Image courtesy of Roberta J. Seidman, MD.
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Hematoxylin and eosin paraffin section shows poly...

Hematoxylin and eosin paraffin section shows polymyositis. Patient had dense endomysial inflammation that contains an abundance of plasma cells, which can be observed in patients with chronic polymyositis. Two necrotic myofibers, characterized by dense eosinophilic staining, are observed. Focal fatty infiltration of the muscle is present in the lower left quadrant of the photomicrograph. Image courtesy of Roberta J. Seidman, MD.

Hematoxylin and eosin paraffin section shows poly...
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Media file 4: Hematoxylin and eosin paraffin section shows polymyositis. Photomicrograph illustrates attack on a nonnecrotic myofiber by autoaggressive T lymphocytes. On the left, the central myofiber is intact. On the right, it is obliterated by a segmental inflammatory attack. If immunohistochemistry were performed, expected findings would include an admixture of CD8 T lymphocytes and macrophages in the inflammatory process. Image courtesy of Roberta J. Seidman, MD.
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Hematoxylin and eosin paraffin section shows poly...

Hematoxylin and eosin paraffin section shows polymyositis. Photomicrograph illustrates attack on a nonnecrotic myofiber by autoaggressive T lymphocytes. On the left, the central myofiber is intact. On the right, it is obliterated by a segmental inflammatory attack. If immunohistochemistry were performed, expected findings would include an admixture of CD8 T lymphocytes and macrophages in the inflammatory process. Image courtesy of Roberta J. Seidman, MD.

Hematoxylin and eosin paraffin shows dermatomyosi...
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Media file 5: Hematoxylin and eosin paraffin shows dermatomyositis. In dermatomyositis, inflammation is characteristically perivascular and perimysial. Vessel oriented approximately vertically in the center has a mild perivascular chronic inflammatory infiltrate. The endothelium is plump. The wall is not necrotic. A few lymphocytes in the wall of the vessel are probably in transit from the lumen to the external aspect of the vessel. Some observers may interpret this finding as vasculitis, but it is certainly neither necrotizing vasculitis nor arteritis. Image courtesy of Roberta J. Seidman, MD.
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Hematoxylin and eosin paraffin shows dermatomyosi...

Hematoxylin and eosin paraffin shows dermatomyositis. In dermatomyositis, inflammation is characteristically perivascular and perimysial. Vessel oriented approximately vertically in the center has a mild perivascular chronic inflammatory infiltrate. The endothelium is plump. The wall is not necrotic. A few lymphocytes in the wall of the vessel are probably in transit from the lumen to the external aspect of the vessel. Some observers may interpret this finding as vasculitis, but it is certainly neither necrotizing vasculitis nor arteritis. Image courtesy of Roberta J. Seidman, MD.

Hematoxylin and eosin frozen section shows perifa...
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Media file 6: Hematoxylin and eosin frozen section shows perifascicular atrophy in dermatomyositis. Fascicles in this sample show atrophy, predominantly at the periphery, along the connective-tissue border. Ischemia is considered to cause perifascicular atrophy. This finding is characteristic of dermatomyositis, mostly associated with the juvenile form but it is also observed in the adult form. Image courtesy of Roberta J. Seidman, MD.
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Hematoxylin and eosin frozen section shows perifa...

Hematoxylin and eosin frozen section shows perifascicular atrophy in dermatomyositis. Fascicles in this sample show atrophy, predominantly at the periphery, along the connective-tissue border. Ischemia is considered to cause perifascicular atrophy. This finding is characteristic of dermatomyositis, mostly associated with the juvenile form but it is also observed in the adult form. Image courtesy of Roberta J. Seidman, MD.

Immunofluorescence for membrane attack complex of...
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Media file 7: Immunofluorescence for membrane attack complex of complement (MAC) in dermatomyositis. Bright ring of yellow-green fluorescence at the center represents MAC in the wall of the microvessel. Finding was not present after treatment with steroids. Image courtesy of Roberta J. Seidman, MD.
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Immunofluorescence for membrane attack complex of...

Immunofluorescence for membrane attack complex of complement (MAC) in dermatomyositis. Bright ring of yellow-green fluorescence at the center represents MAC in the wall of the microvessel. Finding was not present after treatment with steroids. Image courtesy of Roberta J. Seidman, MD.

More on Dermatomyositis/Polymyositis

Overview: Dermatomyositis/Polymyositis
Differential Diagnoses & Workup: Dermatomyositis/Polymyositis
Treatment & Medication: Dermatomyositis/Polymyositis
Follow-up: Dermatomyositis/Polymyositis
Multimedia: Dermatomyositis/Polymyositis
References
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References

  1. Na SJ, Kim SM, Sunwoo IN, Choi YC. Clinical characteristics and outcomes of juvenile and adult dermatomyositis. J Korean Med Sci. Aug 2009;24(4):715-21. [Medline].

  2. Hengstman GJ, van den Hoogen FH, van Engelen BG. Treatment of the inflammatory myopathies: update and practical recommendations. Expert Opin Pharmacother. May 2009;10(7):1183-90. [Medline].

  3. Chérin P. [Current therapy for polymyositis and dermatomyositis]. Rev Med Interne. Jun 2008;29 Spec No 2:9-14. [Medline].

  4. [Guideline] Elovaara I, Apostolski S, van Doorn P, Gilhus NE, Hietaharju A, Honkaniemi J, et al. EFNS guidelines for the use of intravenous immunoglobulin in treatment of neurological diseases: EFNS task force on the use of intravenous immunoglobulin in treatment of neurological diseases. Eur J Neurol. Sep 2008;15(9):893-908. [Medline].

  5. Danieli MG, Calcabrini L, Calabrese V, Marchetti A, Logullo F, Gabrielli A. Intravenous immunoglobulin as add on treatment with mycophenolate mofetil in severe myositis. Autoimmun Rev. Apr 19 2009;[Medline].

  6. Banker BQ, Engel AG. The polymyositis and dermatomyositis syndrome. In: Myology. New York, NY:. McGraw-Hill;1986:1385-422.

  7. Bombardieri S, Hughes GR, Neri R, et al. Cyclophosphamide in severe polymyositis. Lancet. May 20 1989;1(8647):1138-9. [Medline].

  8. Cervera R, Ramirez G, Fernandez-Sola J, et al. Antibodies to endothelial cells in dermatomyositis: association with interstitial lung disease. BMJ. Apr 13 1991;302(6781):880-1. [Medline].

  9. Cherin P, Herson S, Wechsler B, et al. Efficacy of intravenous gammaglobulin therapy in chronic refractory polymyositis and dermatomyositis: an open study with 20 adult patients. Am J Med. Aug 1991;91(2):162-8. [Medline].

  10. Dalakas M. Treatment of polymyositis and dermatomyositis. Curr Opin Rheumatol. Dec 1989;1(4):443-9. [Medline].

  11. Dalakas MC, Illa I, Dambrosia JM, et al. A controlled trial of high-dose intravenous immune globulin infusions as treatment for dermatomyositis. N Engl J Med. Dec 30 1993;329(27):1993-2000. [Medline].

  12. Dalakas MC, Rowland LP, DiMauro S. Inflammatory myopathies. In: Vinken PJ, Bruyn GW, Uchio E, eds. Handbook of Clinical Neurology. Vol 18. New York, NY:. Elsevier Science Inc;1992:369-90.

  13. Emslie-Smith AM, Engel AG. Microvascular changes in early and advanced dermatomyositis: a quantitative study. Ann Neurol. Apr 1990;27(4):343-56. [Medline].

  14. Engel AG, Hohlfeld R, Banker BQ. The polymyositis and dermatomyositis syndrome. In: Myology. New York, NY:. McGraw-Hill;1994:1335-83.

  15. Grau JM, Herrero C, Casademont J, et al. Cyclosporine A as first choice therapy for dermatomyositis. J Rheumatol. Feb 1994;21(2):381-2. [Medline].

  16. Illa I, Dinsmore S, Dalakas MC. Immune-mediated mechanisms and immune activation of fibroblasts in the pathogenesis of eosinophilia-myalgia syndrome induced by L-tryptophan. Hum Pathol. Jul 1993;24(7):702-9. [Medline].

  17. Koski CL, Patterson JV. Intravenous immunoglobulin use for neurologic diseases. J Infus Nurs. May-Jun 2006;29(3 Suppl):S21-8. [Medline].

  18. Lang BA, Laxer RM, Murphy G, et al. Treatment of dermatomyositis with intravenous gammaglobulin. Am J Med. Aug 1991;91(2):169-72. [Medline].

  19. Marinelli WA, Leatherman JW. Neuromuscular disorders in the intensive care unit. Crit Care Clin. Oct 2002;18(4):915-29, x. [Medline].

  20. Miller FW, Leitman SF, Cronin ME, et al. Controlled trial of plasma exchange and leukapheresis in polymyositis and dermatomyositis. N Engl J Med. May 21 1992;326(21):1380-4. [Medline].

  21. Otero C, Dalakas MC. Onset of polymyositis (PM) and dermatomyositis (DM) during pregnancy: report of 4 cases. Ann Neurol. 1992;32:282.

  22. Pellissier JF, Civatte M, Fernandez C, et al. [Dermatomyositis and polymyositis]. Rev Neurol (Paris). Oct 2002;158(10 Pt 1):934-47. [Medline].

  23. Rowland LP. Polymyositis, inclusion body myositis, and related myopathies. In: Merritt's Neurology. 10th ed. Philadelphia, Pa:. Lippincott Williams & Wilkins;2000:765-9.

  24. Stein DP, Dalakas MC. Intercellular adhesion molecule-1 expression is up regulated in patients with dermatomyositis (DM) [abstract]. Ann Neurol. 1993;34:268.

  25. Targoff IN. Immune mechanisms in myositis. Curr Opin Rheumatol. Dec 1990;2(6):882-8. [Medline].

  26. Targoff IN, Reichlin M. Measurement of antibody to Jo-1 by ELISA and comparison to enzyme inhibitoryactivity. J Immunol. May 1 1987;138(9):2874-82. [Medline].

  27. Whitaker JN, Engel WK. Vascular deposits of immunoglobulin and complement in idiopathic inflammatory myopathy. N Engl J Med. Feb 17 1972;286(7):333-8. [Medline].

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Further Reading

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Keywords

dermatomyositis, polymyositis, inflammatory myopathies, primary muscle weakness, endomysial inflammation, elevated levels of serum muscle enzymes, myositis-associated antibodies, MAA, myositis-specific antibodies, MSA, muscle diseases, juvenile dermatomyositis, childhood dermatomyositis, overlap syndrome, polymyositis associated with neoplasia, polymyositis associated with connective tissue disorder, dermatomyositis associated with neoplasia, childhood dermatomyositis with necrotizing vasculitis, childhood myositis with necrotizing vasculitis

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Contributor Information and Disclosures

Author

Sushma Podila, MD, Resident Physician, Department of Neurology, Columbia Presbyterian Medical Center
Disclosure: Nothing to disclose.

Coauthor(s)

Thomas H Brannagan III, MD, Associate Professor of Clinical Neurology and Director, Peripheral Neuropathy Center, Columbia University, College of Physicians and Surgeons; Co-Director, EMG Laboratory, New York-Presbyterian Hospital, Columbia Campus, New York
Thomas H Brannagan III, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and Peripheral Nerve Society
Disclosure: Nothing to disclose.

Medical Editor

Milind J Kothari, DO, Professor and Vice-Chair, Department of Neurology, Pennsylvania State University College of Medicine; Consulting Staff, Department of Neurology, Hershey Medical Center
Milind J Kothari, DO is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and American Neurological Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Glenn Lopate, MD, Associate Professor, Department of Neurology, Division of Neuromuscular Diseases, Washington University School of Medicine; Chief of Neurology, St Louis ConnectCare, Consulting Staff, Barnes Jewish Hospital
Glenn Lopate, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and Phi Beta Kappa
Disclosure: Nothing to disclose.

CME Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Nicholas Y Lorenzo, MD, Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants
Nicholas Y Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Neurology
Disclosure: Nothing to disclose.

 
 
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