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Skin Involvement in Juvenile Dermatomyositis Is Associated with Loss of End Row Nailfold
Capillary Loops
RACHEL LYNNE SMITH, JOYCE SUNDBERG, ELI SHAMIYAH, ALAN DYER, and LAUREN M. PACHMAN
ABSTRACT. Methods. Sixty newly diagnosed, previously untreated children who met the Bohan-Peter criteria for definite JDM were seen between 1993 and 2002. They were classified by duration of untreated disease and by a disease activity score (DAS) composed of separate subscores for dermatological (DAS skin) and musculoskeletal (DAS muscle) findings. Routine NFC measurements yielded the number of end row loops, arboreal (bushy), and dilated capillary loops. Laboratory testing included muscle enzymes, von Willebrand Factor Antigen, and neopterin. Results. DAS skin, but not DAS muscle, was associated with NFC end row capillary loss (rs = –0.394, p = 0.008). End row capillary loss (reflecting avascularity), arboreal (bushy), and dilated capillary loops (reflecting change in vascular morphology) were each associated with longer untreated symptom duration (rs = –0.401, rs = 0.534, rs = 0.371). Conclusion. End row capillary loss measured by NFC was associated with the dermatological, but not musculoskeletal manifestations of JDM, suggesting that damage to skin and muscle may each have distinct disease pathophysiology. In JDM, skin involvement indicates a vasculopathy that progresses with increasing duration of untreated disease and is not revealed by standard serological laboratory tests. We propose that the cutaneous manifestations of JDM are associated with vascular disease and warrant aggressive therapy. (J Rheumatol 2004;31:1644-9) Key Indexing Terms:
NAILFOLD CAPILLAROSCOPY
From the Department of Pediatrics, Division of Immunology/ Rheumatology, Northwestern University Feinberg School of Medicine; and the Department of Pediatrics, Division of Immunology/Rheumatology, Children's Memorial Hospital, Chicago, Illinois, USA. Supported by the National Arthritis Foundation, Daniel J. Pachman and Vivian A. Pachman Dermatomyositis Research Fund, Marlene Apflebaum Foundation, and the Jack Pappas Fund. Dr. Smith is the recipient of an American College of Rheumatology Medical Student Award, 2002. R.L. Smith, MD, Northwestern University Feinberg School of Medicine; J. Sundberg, RN, Northwestern University Feinberg School of Medicine, Department of Pediatrics, Division of Immunology/Rheumatology, Children's Memorial Hospital; E. Shamiyah, MS, Department of Medicine, Northwestern University Feinberg School of Medicine; A. Dyer, PhD, Department of Preventive Medicine, Northwestern University Feinberg School of Medicine; L.M. Pachman, MD, Department of Pediatrics, Division of Immunology/Rheumatology, Northwestern University Feinberg School of Medicine. Address reprint requests to Dr. L.M. Pachman, Children's Memorial Medical Center, Box 212, 2300 Children's Plaza, Chicago, IL 60614. E-mail: pachman@northwestern.edu Submitted September 17, 2003; revision accepted February 13, 2004. |