Skin involvement in juvenile dermatomyositis is associated with loss of end row nailfold capillary loops

Children's Memorial Hospital, Chicago, Illinois, United States
The Journal of Rheumatology (Impact Factor: 3.19). 09/2004; 31(8):1644-9.
Source: PubMed


To determine associations of dermatological findings in children with juvenile dermatomyositis (JDM) with specific nailfold capillary (NFC) structural abnormalities.
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.
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).
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.

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    • "Previous studies have confirmed the observation that prolonged duration of untreated symptoms in children with JDM are highly associated with the development of pathologic calcifications, one of the most debilitating complications of JDM [22,23]. Untreated children with a longer duration of chronic symptoms had a higher frequency of loss of nailfold capillary end row loops [24], which persisted after 36 months of therapy [25]. The chronic inflammation associated with nailfold capillary loss was accompanied by impaired absorption of orally administered corticosteroids [26]. "
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    • "Such a categorization has permitted our group to observe associations between disease activity in the specific tissue compartments and laboratory and/or clinical measurements. For example, we have recently reported that abnormal nailfold capillary blood vessels are associated with DAS skin but not DAS muscle [5]. Conversely, a decrease in the absolute count of circulating natural killer (NK) cells is associated with DAS muscle but not DAS skin [6]. "
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