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ISSN 0210-5187 (Electronic)

2016, Number 2

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Med Cutan Iber Lat Am 2016; 44 (2)

Dermatomyositis: Presentation of 16 cases and review of the literature

Ibañez MJ, Bolomo G, Gómez SMS, Miraglia E, Arena G, Chiavassa AM

Language: Spanish
References: 29
Page: 110-122
PDF size: 565.84 Kb.


ABSTRACT

Introduction: Dermatomyositis is an infrequent connective tissue disease that involves skin, muscle and internal organs. Sontheimer et al (2002) included it into the idiopathic inflammatory myopathies and subdivided the juvenile and the adult forms in two groups: classic and clinically amiopathic, related to the presence or absence of muscular symptomatology. The cutaneous manifestations of dermatomyositis are the same for all its clinical forms, which leads us to study every case exhaustively with the aim to exclude internal neoplasia, visceral involment and concomitant colagenopathy. Objective: Identify the different clinical variants of dermatomyositis, establish distribution by gender and age, describe their associations, and compare the results with the literature. Material and methods: A retrospective observational study of 16 adults patients, 12 women and 4 men, who consulted in the Dermatology Department of HIGA San Martin of La Plata, Buenos Aires, Argentina, during the period between June 2010 and June 2015 was performed. Results: In our serie we found 68.75% of the classical form and 31.25% of the clinically amiopathic dermatomyositis (60% amyopathic and 40% hipomiophatic), and 25% from all cases was associated with internal malignancy, with predominance in females. In 81.25% of the patients we recognized pathognomonic cutaneous lesions, 100% had some type of characteristic lesion of dermatomyositis and only 6.25% had compatible lesions. The most constant skin manifestation presented in all patients was the violaceous macular rash. We identify 3 unusual forms of dermatomyositis: flagellated, poikiloderma and Wong’s dermatomyositis. The systemic treatments instituted were: oral corticosteroids (meprednisone) in 87.5% (n = 14), hydroxychloroquine in 56.25% (n = 9), methotrexate 56.25% (n = 9), azathioprine in 37.5% (n = 6), mycophenolate mofetil in 31.25% (n = 5), pulse methylprednisolone in 25% (n = 4), cyclophosphamide pulses at 12.5% (n = 2), rituximab in 12.5% (n = 2) and intravenous immunoglobulin at 6.25% (n = 1). Four cases died, 2 of them (50%) due to an internal malignancy, one (25%) to respiratory failure secondary to muscle commitment of dermatomyositis and one (25%) due to a causes unrelated to it. Conclusions: This study allows knowing the composition of our population of dermatomyositis, as the prevalence of the different variants of its association with internal neoplasia and commitment of interstitial lung disease, the presence of specific antibodies such as anti Jo-1, the instituted treatment and the causes of death.


REFERENCES

  1. Candiz ME. Dermatomiositis asociada a neoplasias. Dermatol Argent. 2012; 17 (6): 434-440.

  2. Hassan ML, Capecce A, Melloni ME, Saposnik M, Ruzzi I, Gramajo J et al. Estudio retrospectivo de la dermatomiositis. análisis de 40 casos de consulta en la División de Dermatología. Dermatol Argent. 2009; 15 (1): 27-36.

  3. Gerami P, Schope JM, McDonald L, Walling HW, Sontheimer RD. A systematic review of adult-onset clinically amyopathic dermatomyositis (dermatomyositis sine myositis): a missing link within the spectrum of the idiopathic inflammatory myopathies. J Am Acad Dermatol. 2006; 54 (4): 597-613.

  4. Bielsa-Marsol I. Dermatomiositis. Reumatol Clin. 2009; 5 (5): 216-222.

  5. Bielsa-Marsol I. Nuevos autoanticuerpos en la dermatomiositis. Actas Dermosifiliogr. 2009; 100 (3): 182-189.

  6. Schultz HY, Dutz JP, Furukawa F, Goodfield MJ, Kuhn A, Lee LA et al. From pathogenesis, epidemiology, and genetics to definitions, diagnosis, and treatments of cutaneous lupus erythematosus and dermatomyositis: a report from the 3rd International Conference on Cutaneous Lupus Erythematosus (ICCLE) 2013. J Invest Dermatol. 2015; 135 (1): 7-12.

  7. De Luca D, Staiger H, Baztán MC, Enz P, Kowalczuk A, Galimberti R. Síndrome antisintetasa. Rev Asoc Col Dermatol. 2012; 20 (4): 381-384.

  8. Ghirardello A, Borella E, Beggio M, Franceschini F, Fredi M, Doria A. Myositis autoantibodies and clinical phenotypes. Auto Inmun Highlights. 2014; 5 (3): 69-75.

  9. Lu X, Yang H, Shu X, Chen F, Zhang Y, Zhang S et al. Factors predicting malignancy in patients with polymyositis and dermatomyostis: a systematic review and meta-analysis. PLoS One. 2014; 9 (4): e94128.

  10. Saettone-León A. Dermatosis paraneoplásicas. Dermatol Perú. 2012; 22 (4): 149-160.

  11. Haro R, Revelles JM, Fariña MDC, Martín L, Requena L. Wong’s dermatomyositis: a new case and review of the literature. Int J Dermatol. 2013; 52 (4): 466-470.

  12. Wong KO. Dermatomyositis: a clinical investigation of twenty-three cases in Hong Kong. Br J Dermatol. 1969; 81 (7): 544-547.

  13. Brunsting LA, Christianson HB, Perry HO. Dermatomyositis; unusual features, complications, and treatment. AMA Arch Derm. 1956; 74 (6): 581-589.

  14. O’Leary P. Dermatomyositis. Arch Dermatol. 1953; 68: 101-102.

  15. Aguilar AR, Gomez F, Balsa FT, Framil JP, Oubina PN. Pityriasis rubra pilaris with muscle and joint involvement. Dermatologica. 1973; 146 (6): 361-366.

  16. Caporali R, Cavagna L, Bellosta M, Bogliolo L, Montecucco C. Inflammatory myopathy in a patient with cutaneous findings of pityriasis rubra pilaris: a case of Wong’s dermatomyositis. Clin Rheumatol. 2004; 23 (1): 63-65.

  17. Ortonne N, Viguier M, Vignon-Pennamen MD, Morel P, Janin A. Myosite arrectopilaire au cours d’une dermatomyosite: un signe histologique inhabituel. Ann Pathol. 2002; 22 (1): 39-42.

  18. Canavan T, Sidorsky T, Doan LT, Ricardo-Gonzalez RR, Shen G, Rosenblum MD. A case of Wong-type dermatomyositis with concomitant anti-MDA5 features. J Am Acad Dermatol. 2014; 3 (70): e62-e64.

  19. Beckum KM, Gavino AC, Marks S, Andea AA, Elewski BE. Wong type dermatomyositis: 20th case reported. J Drugs Dermatol. 2010; 9 (12): 1475-1476.

  20. de la Tribonnière X, Delaporte E, Alfandari S, Rowland-Payne CM, Piette F, Bergoend H. Dermatomyositis with follicular hyperkeratosis. Dermatology. 1995; 191 (3): 242-244.

  21. Dupré A, Floutard M, Christol B, Rumeau H. Dermatomyositis with spinulosis (Wong type dermatomyositis). Histologic study. Attempt at pathogenic interpretation. Ann Dermatol Syphiligr (Paris). 1976; 103 (2): 141-149.

  22. Granier H, Bressollette L, Morand C, Gavanou J, Mottier D. Dermatomyositis type Wong in a 50-year-old woman. Ann Med Interne (Paris). 1991; 142 (5): 389.

  23. Lupton JR, Figueroa P, Berberian BJ, Sulica VI. An unusual presentation of dermatomyositis: the type Wong variant revisited. J Am Acad Dermatol. 2000; 43 (5 Pt 2): 908-912.

  24. Polat M, Lenk N, Ustün H, Oztaş P, Artüz F, Alli N. Dermatomyositis with a pityriasis rubra pilaris-like eruption: an uncommon cutaneous manifestation in dermatomyositis. Pediatr Dermatol. 2007; 24 (2): 151-154.

  25. Requena L, Grilli R, Soriano L, Escalonilla P, Fariña C, Martín L. Dermatomyositis with a pityriasis rubra pilaris-like eruption: a little-known distinctive cutaneous manifestation of dermatomyositis. Br J Dermatol. 1997; 136 (5): 768-771.

  26. Betancur JF, Jiménez DF, Bonilla-Abadía F, Tobón GJ. Compromiso hepático de las enfermedades autoinmunes sistémicas. Rev Colomb Reumatol. 2015; 22 (1): 47-58.

  27. Gómez M, Urquijo P, Mela M, Pittana P. Capilaroscopia periungueal. Arch Argent Dermatol. 2011; 61: 197-202.

  28. Sánchez-Fernández SA, Fernández JAC, Vargas LMR. Eficacia de rituximab en dermatomiositis y polimiositis refractarias al tratamiento convencional. Reumatol Clin. 2013; 9 (2): 117-119.

  29. Enk A. Guidelines on the use of high-dose intravenous immunoglobulin in dermatology. Eur J Dermatol. 2008; 19 (1): 90-98.




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