A Case of Dermatomyositis: Diagnostic Challenge in Low Resource Settings
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Submitted
July 22, 2020
Published
July 22, 2020
Keywords:
-
Dermatomyositis, heliotrope rash, gottron’s papules
Abstract
Dermatomyositis is one of the idiopathic inflammatory myopathies. It is characterized clinically by progressive symmetrical proximal muscle weakness and a characteristic rash. Although the process primarily attacks the skin and the muscles, it is a systemic disease with frequent manifestations in the gastrointestinal tract and pulmonary system. Dermatomyositis has been linked to internal malignancy in somewhere between 15% and 25%. We report a case involving a 30 years old female nurse who presented with extensive erythema involving face and upper neck, and deteriorating proximal muscular weakness for three months. Later muscle enzymes, EMG and biopsy were done from BSMMU, Dhaka, confirmed the diagnosis of Dermatomyositis. Oral prednisolone along with azathioprine, hydroxychloroquine and photo protection with sun screen were started and showed good response both clinically and biochemically.
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References
1. Callen JP. Dermatomyositis. Lancet 2000;355:53 - 7.
2. Plotz PH, Rider LG, Targoff IN, Raben N, O’Hanlon TP, Miller FW. Myositis: immunologic contributions to understanding cause, pathogenesis,
and therapy. Ann Intern Med 1995;122:715- 24.
3. Targoff IN. Dermatomyositis and polymyositis. Curr Probl Dermatol
1991;3:131 - 80.
4. Bohan A, Peter JB. Polymyositis and dermatomyositis. N Engl J Med
1975;292:344 - 7 and 403- 7 [two part article].
5. Kuru S, Inukai A, Liang Y, Doyu M, Takano A, Sobue G. Tumor necrosis factor–a expression in muscles of polymyositis and dermatomyositis. Acta Neuropathol 2000;99:585- 8.
6. Wanchu A, Khullar M, Sud A, Bambery P. Nitric oxide production is
increased in patient with inflammatory myositis. Nitric Oxide 1999; 3:454- 8.
7. Shimizu T, Tomita Y, Son K, Nishinarita S, Sawada S, Horie T. Elevation of serum soluble tumor necrosis factor receptors in patients with polymyositis and dermatomyositis. Clin Rheumatol 2000;19: 352- 9.
8. Sugugiura T, Kawaguchi Y, Harigai M, et al. Increased CD40 expression on muscle cells of polymyositis and dermatomyositis: role of CD40-CD40 ligand interaction in IL-6, IL-8, IL-5, and monocyte chemoattractant protein–1 production. J Immunol 2000; 164:6593- 600.
9. Nyberg P, Wikman A-L, Nennesmo I, Lundberg I. Increased expression of interleukin 1a and MHC class I in muscle tissue of patients with chronic, inactive polymyositis and dermatomyositis. J Rheumatol 2000;27:940 - 8.
10. William D. James, Timothy G. Berger, Dirk M. Eloston,. Andrews's diseases of the skin. 11th edition 2011, 165-168.
11. M.J.D. Goodfield, S.K. Jones and D.J.Veale, the 'Connective Tissue Diseases'. Rook's Textbook of Dermatology.8thedition ,2010;51.120- 51.129
12. Brain R Walker, Nicki R Colledge, Stuart H Relston, Ian D Penman. Davidson’s Principles and Practice of Medicine. 22nd ed. Elsevier limited; 2014. p.1114.
13. Bohan A, Peter JB, Bowman RL, Pearson CM. A computer-assisted analysis of 153 patients with polymyositis and dermatomyositis. Medicine 1977;56:255 - 86.
14. Euwer RL, Sontheimer RD. Amyopathic DM (DM sine´ myositis). J Am Acad Dermatol 1991;24:959- 66.
15. Sayers ME, Chou SM, Calabrese LH. Inclusion body myositis: analysis of 32 cases. J Rheumatol 1992;19:1385- 9.
16. Calabrese LH, Chou SM. Inclusion body myositis. Rheum Dis Clin North Am 1994;20:955- 72.
17. Dourmishev AL, Dourshimev LA. Dermatomyositis and drugs. Adv Exp Med Biol 1999;445:187 - 91.
18. SontheimerRD.Would a new name hasten the acceptance of amyopathic dermatomyositis (dermatomyositis sine myositis) as a distinctive subset within the idiopathic inflammatory dermatomyopathies spectrum of clinical illness? J Am Acad Dermatol 2002;46:626-36.
19. Adriana Maluf Elias Sallum, Maria Helena Bittencurt Kiss, Silvana Sachetti, Maria Bernadate Dutra Resende, Kelly Cristina Moutinho, Mary de Souza Carvalho, Clovis Arthur Almeida Silva, Suely Kazue NagahashiMarie.Juvenile dermattomyositis: clinical, laboratorial, histological, therapeutical and evolution parameters of 35 patients Arq Neuropsiquiatr 2002;60(4): 889-899.
20. Woo TR, Rasmussen J, Callen JP. Recurrent photosensitive dermatitis preceding juvenile dermatomyositis. Pediatr Dermatol 1985;2:207- 12.
21. Callen JP. Photodermatitis in a 6-year-old child. Arthritis Rheum 1993;36:1483- 5.
22. Zuber M, John S, Pfreundschuh M, Gause A. A young woman with a photosensitive pruritic rash on her face and upper trunk. Arthritis Rheum 1996;39:1419- 22.
23. Callen JP. Photosensitivity in collagen vascular diseases. Semin Cutan Med Surg 1999;18:293 -6.
24. Kasteler JS, Callen JP. Scalp involvement in dermatomyositis. Often overlooked or misdiagnosed. JAMA 1994;272:1939 - 41.
25. Nitsche A, San Agustin PG, Amado V. Prina AP, Corsaro G. Trunk and Abdominal wall edema in dermatomyositis. Medicina (B Aires). 1988; 48(3):331-2. [PMID: 2977631]
26. Chai Y, Bertorini TE, Li YD, Mitchell C, Guan H. Limb edema and anasarca associated with severe dermatomyositis: report of four cases. Neuromuscul Disord. 2011 Jun;21(6):439-42. [PMID: 21482469]
27. Andonopoulos AP, Gogos CA, Tzanakakis G. Subcutaneous Edema: an unrecognised feature of acute polymyositis. Rheumatol Int. 1993; 13(4):159-161. [PMID: 8310209]
28. Somani, AK, Swick A, Cooper K. McCormick TS. Severe Dermatomyositis Triggered by Interferon Beta-1a Therapy and Associated With Enhanced Type I Interferon Signalling. Arch Dermatol. 2008 Oct; 144(10): 1341-1349. [PMID: 18936398]
29. Bohan A, Peter JB, Polymyositis and dermatomyositis (first of two parts). N Engl J Med. 1975 1975 Feb 13;292(7):344-7. [PMID: 1090839]
30. Werner de Castro GR, Appenzeller S, Bértolo MB, Costallat LT. Acute dermatomyositis with subcutaneous generalized edema. Clinical Rheumatology, 2006 Nov, 25(6):898-900. [PMID: 16308667]
31. Lee KH, Lim SR, Kim YJ, Lee KJ, Myung DS, Jeong HC, Yoon W, Lee SS, Park YW. Acute dermatomyositis associated with generalized subcutaneous edema. Rheumatol Int, 2008 Jun; 28(8) :797-800. [PMID: 18193426]
32. Haroon M, Eltahir A, Harney S. Generalized subcutaneous edema as a rare manifestation of dermatomyositis: clinical lesson from a rare feature. J Clin Rheumatol. 2011 April;17(3):135-7. [PMID: 21441819]
33. Jung KD, Kim PS, Park HY, Kim CR, Byun JY, Lee DY, Lee JH, Yang JM, Lee ES. Dermatomyositis associated with generalized subcutaneous edema and Evans syndrome. J Am Acad Dermatol. 2012 Jan ;66(1):144-7. [PMID: 22030016]
34. Noss EH, Hausner-Sypek DL, Weinblatt ME, Rituximab as therapy for refractory polymyositis and dermatomyositis. J Rheumatol. 2006 May; 33:1021–1026. [PMID: 16541475]
35. Rios Fernandez R, Callejas Rubio JL, Sanchez Cano D, Sáez Moreno JA, Ortego Centeno N. Rituximab in the treatment of dermatomyositis and other inflammatory myopathies. A report of 4 cases and review of the literature. Clin Exp Rheumatol. 2009 Nov-Dec; 27(6):1009-16. [PMID: 20149324]
2. Plotz PH, Rider LG, Targoff IN, Raben N, O’Hanlon TP, Miller FW. Myositis: immunologic contributions to understanding cause, pathogenesis,
and therapy. Ann Intern Med 1995;122:715- 24.
3. Targoff IN. Dermatomyositis and polymyositis. Curr Probl Dermatol
1991;3:131 - 80.
4. Bohan A, Peter JB. Polymyositis and dermatomyositis. N Engl J Med
1975;292:344 - 7 and 403- 7 [two part article].
5. Kuru S, Inukai A, Liang Y, Doyu M, Takano A, Sobue G. Tumor necrosis factor–a expression in muscles of polymyositis and dermatomyositis. Acta Neuropathol 2000;99:585- 8.
6. Wanchu A, Khullar M, Sud A, Bambery P. Nitric oxide production is
increased in patient with inflammatory myositis. Nitric Oxide 1999; 3:454- 8.
7. Shimizu T, Tomita Y, Son K, Nishinarita S, Sawada S, Horie T. Elevation of serum soluble tumor necrosis factor receptors in patients with polymyositis and dermatomyositis. Clin Rheumatol 2000;19: 352- 9.
8. Sugugiura T, Kawaguchi Y, Harigai M, et al. Increased CD40 expression on muscle cells of polymyositis and dermatomyositis: role of CD40-CD40 ligand interaction in IL-6, IL-8, IL-5, and monocyte chemoattractant protein–1 production. J Immunol 2000; 164:6593- 600.
9. Nyberg P, Wikman A-L, Nennesmo I, Lundberg I. Increased expression of interleukin 1a and MHC class I in muscle tissue of patients with chronic, inactive polymyositis and dermatomyositis. J Rheumatol 2000;27:940 - 8.
10. William D. James, Timothy G. Berger, Dirk M. Eloston,. Andrews's diseases of the skin. 11th edition 2011, 165-168.
11. M.J.D. Goodfield, S.K. Jones and D.J.Veale, the 'Connective Tissue Diseases'. Rook's Textbook of Dermatology.8thedition ,2010;51.120- 51.129
12. Brain R Walker, Nicki R Colledge, Stuart H Relston, Ian D Penman. Davidson’s Principles and Practice of Medicine. 22nd ed. Elsevier limited; 2014. p.1114.
13. Bohan A, Peter JB, Bowman RL, Pearson CM. A computer-assisted analysis of 153 patients with polymyositis and dermatomyositis. Medicine 1977;56:255 - 86.
14. Euwer RL, Sontheimer RD. Amyopathic DM (DM sine´ myositis). J Am Acad Dermatol 1991;24:959- 66.
15. Sayers ME, Chou SM, Calabrese LH. Inclusion body myositis: analysis of 32 cases. J Rheumatol 1992;19:1385- 9.
16. Calabrese LH, Chou SM. Inclusion body myositis. Rheum Dis Clin North Am 1994;20:955- 72.
17. Dourmishev AL, Dourshimev LA. Dermatomyositis and drugs. Adv Exp Med Biol 1999;445:187 - 91.
18. SontheimerRD.Would a new name hasten the acceptance of amyopathic dermatomyositis (dermatomyositis sine myositis) as a distinctive subset within the idiopathic inflammatory dermatomyopathies spectrum of clinical illness? J Am Acad Dermatol 2002;46:626-36.
19. Adriana Maluf Elias Sallum, Maria Helena Bittencurt Kiss, Silvana Sachetti, Maria Bernadate Dutra Resende, Kelly Cristina Moutinho, Mary de Souza Carvalho, Clovis Arthur Almeida Silva, Suely Kazue NagahashiMarie.Juvenile dermattomyositis: clinical, laboratorial, histological, therapeutical and evolution parameters of 35 patients Arq Neuropsiquiatr 2002;60(4): 889-899.
20. Woo TR, Rasmussen J, Callen JP. Recurrent photosensitive dermatitis preceding juvenile dermatomyositis. Pediatr Dermatol 1985;2:207- 12.
21. Callen JP. Photodermatitis in a 6-year-old child. Arthritis Rheum 1993;36:1483- 5.
22. Zuber M, John S, Pfreundschuh M, Gause A. A young woman with a photosensitive pruritic rash on her face and upper trunk. Arthritis Rheum 1996;39:1419- 22.
23. Callen JP. Photosensitivity in collagen vascular diseases. Semin Cutan Med Surg 1999;18:293 -6.
24. Kasteler JS, Callen JP. Scalp involvement in dermatomyositis. Often overlooked or misdiagnosed. JAMA 1994;272:1939 - 41.
25. Nitsche A, San Agustin PG, Amado V. Prina AP, Corsaro G. Trunk and Abdominal wall edema in dermatomyositis. Medicina (B Aires). 1988; 48(3):331-2. [PMID: 2977631]
26. Chai Y, Bertorini TE, Li YD, Mitchell C, Guan H. Limb edema and anasarca associated with severe dermatomyositis: report of four cases. Neuromuscul Disord. 2011 Jun;21(6):439-42. [PMID: 21482469]
27. Andonopoulos AP, Gogos CA, Tzanakakis G. Subcutaneous Edema: an unrecognised feature of acute polymyositis. Rheumatol Int. 1993; 13(4):159-161. [PMID: 8310209]
28. Somani, AK, Swick A, Cooper K. McCormick TS. Severe Dermatomyositis Triggered by Interferon Beta-1a Therapy and Associated With Enhanced Type I Interferon Signalling. Arch Dermatol. 2008 Oct; 144(10): 1341-1349. [PMID: 18936398]
29. Bohan A, Peter JB, Polymyositis and dermatomyositis (first of two parts). N Engl J Med. 1975 1975 Feb 13;292(7):344-7. [PMID: 1090839]
30. Werner de Castro GR, Appenzeller S, Bértolo MB, Costallat LT. Acute dermatomyositis with subcutaneous generalized edema. Clinical Rheumatology, 2006 Nov, 25(6):898-900. [PMID: 16308667]
31. Lee KH, Lim SR, Kim YJ, Lee KJ, Myung DS, Jeong HC, Yoon W, Lee SS, Park YW. Acute dermatomyositis associated with generalized subcutaneous edema. Rheumatol Int, 2008 Jun; 28(8) :797-800. [PMID: 18193426]
32. Haroon M, Eltahir A, Harney S. Generalized subcutaneous edema as a rare manifestation of dermatomyositis: clinical lesson from a rare feature. J Clin Rheumatol. 2011 April;17(3):135-7. [PMID: 21441819]
33. Jung KD, Kim PS, Park HY, Kim CR, Byun JY, Lee DY, Lee JH, Yang JM, Lee ES. Dermatomyositis associated with generalized subcutaneous edema and Evans syndrome. J Am Acad Dermatol. 2012 Jan ;66(1):144-7. [PMID: 22030016]
34. Noss EH, Hausner-Sypek DL, Weinblatt ME, Rituximab as therapy for refractory polymyositis and dermatomyositis. J Rheumatol. 2006 May; 33:1021–1026. [PMID: 16541475]
35. Rios Fernandez R, Callejas Rubio JL, Sanchez Cano D, Sáez Moreno JA, Ortego Centeno N. Rituximab in the treatment of dermatomyositis and other inflammatory myopathies. A report of 4 cases and review of the literature. Clin Exp Rheumatol. 2009 Nov-Dec; 27(6):1009-16. [PMID: 20149324]
Authors
Gomes, R. R. ., & Khan, D. S. B. . (2020). A Case of Dermatomyositis: Diagnostic Challenge in Low Resource Settings. Journal of Medical Care Research and Review, 3(7), 395–400. https://doi.org/10.15520/mcrr.v3i7.119
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