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Gingival and Periungual Vasculopathy of Juvenile Dermatomyositis

Lisa G. Rider, M.D., and Jane C. Atkinson, D.D.S.

N Engl J Med 2009; 360:e21April 9, 2009

Article

A 16-year-old girl with long-standing juvenile dermatomyositis had an illness marked by intermittent clinical flares, including increased muscle weakness, joint contractures, photosensitive rashes, and dysphagia, despite treatment with multiple immunosuppressive drugs. At presentation, Gottron's papules, a heliotrope rash, and periungual capillary changes were visible, including dilated and tortuous blood vessels with areas of atrophy, telangiectases, and bushy loop formation along the fingernail bed (Panel A, arrows). The patient reported having had bleeding gums that had not responded to traditional dental treatment. Close examination of the mouth revealed markedly dilated capillaries in the attached gingiva, with bushy loop formations that spread over the lower teeth (Panel B, arrows). These areas reflect the vasculopathy associated with dermatomyositis, particularly in its juvenile form. Periungual capillary changes have been reported frequently in patients with juvenile dermatomyositis, whereas the accompanying gingival changes are often present but not frequently recognized. Periungual capillary changes correlate with underlying disease activity, particularly in the skin, and persistent abnormalities are associated with a long course of illness.

Lisa G. Rider, M.D.
Jane C. Atkinson, D.D.S.
National Institutes of Health, Bethesda, MD 20892

Citing Articles (4)

Citing Articles

  1. 1

    Lisa G. Rider, Carol B. Lindsley, James T. Cassidy. 2011. JUVENILE DERMATOMYOSITIS. , 375-413.
    CrossRef

  2. 2

    C. Savioli, C. A. A. Silva, G. M. C. Fabri, K. Kozu, L. M. A. Campos, E. Bonfa, A. M. E. Sallum, J. T. T. de Siqueira. (2010) Gingival capillary changes and oral motor weakness in juvenile dermatomyositis. Rheumatology 49:10, 1962-1970
    CrossRef

  3. 3

    Alessandra Meneghel, Francesco Zulian, Giorgia Martini, Graziella Guariso. (2009) Ischemic Ulcerative Colitis in Juvenile Dermatomyositis. Journal of Pediatric Gastroenterology and Nutrition 49:5, 549
    CrossRef

  4. 4

    Clodagh A Lowry, Clarissa A Pilkington. (2009) Juvenile dermatomyositis: extramuscular manifestations and their management. Current Opinion in Rheumatology 21:6, 575-580
    CrossRef