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

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

[1]  D. Blockmans,et al.  Predictive value of nailfold capillaroscopy in the diagnosis of connective tissue diseases , 1996, Clinical Rheumatology.

[2]  R. Bode,et al.  Disease activity score for children with juvenile dermatomyositis: reliability and validity evidence. , 2003, Arthritis and rheumatism.

[3]  R. Sundel,et al.  Aggressive management of juvenile dermatomyositis results in improved outcome and decreased incidence of calcinosis. , 2002, Journal of the American Academy of Dermatology.

[4]  F. Binaghi,et al.  Nailfold capillaroscopy and laser Doppler flowmetry during standardised cold challenge to evaluate capillary pattern and digital flow in idiopathic acrocyanosis. , 2002, Panminerva medica.

[5]  Eric P. Hoffman,et al.  Gene Expression Profiling in DQA1*0501+ Children with Untreated Dermatomyositis: A Novel Model of Pathogenesis1 , 2002, The Journal of Immunology.

[6]  I. Fresko,et al.  Nailfold capillary abnormalities in patients with familial Mediterranean fever. , 2001, Clinical and experimental rheumatology.

[7]  P. Lachenbruch,et al.  Validation of the Childhood Health Assessment Questionnaire in the juvenile idiopathic myopathies. Juvenile Dermatomyositis Disease Activity Collaborative Study Group. , 2001, The Journal of rheumatology.

[8]  H. Heidrich,et al.  Vital capillary microscopic findings in the nailfold of patients with diabetes mellitus. , 2000, VASA. Zeitschrift fur Gefasskrankheiten.

[9]  L. Pachman,et al.  TNFα-308A allele in juvenile dermatomyositis: Association with increased production of tumor necrosis factor α, disease duration, and pathologic calcifications , 2000 .

[10]  P. Dent,et al.  Juvenile amyopathic dermatomyositis: results of a case finding descriptive survey. , 2000, The Journal of rheumatology.

[11]  R. Bono,et al.  Quantitative nailfold capillary microscopy findings in patients with acrocyanosis compared with patients having systemic sclerosis and control subjects. , 2000, Journal of the American Academy of Dermatology.

[12]  T. Moore,et al.  The influence of age on nailfold capillary dimensions in childhood. , 2000, The Journal of rheumatology.

[13]  L. Pachman,et al.  Intravenous corticosteroids: adverse reactions are more variable than expected in children. , 1998, The Journal of rheumatology.

[14]  M. Ahern,et al.  Decreased nailfold capillary density in limited scleroderma with pulmonary hypertension. , 1998, Asian Pacific journal of allergy and immunology.

[15]  A. Paller,et al.  Juvenile dermatomyositis presenting with rash alone. , 1997, Pediatrics.

[16]  N. Pangratis.  Diagnostic investigation using vital capillary microscopy and dynamic capillaroscopy. , 1997, Clinical hemorheology and microcirculation.

[17]  E. Silverman,et al.  Measuring disability in juvenile dermatomyositis: validity of the childhood health assessment questionnaire. , 1995, The Journal of rheumatology.

[18]  L. Pachman.  Imperfect indications of disease activity in juvenile dermatomyositis. , 1995, The Journal of rheumatology.

[19]  A. Martini,et al.  Correlation of serum neopterin concentrations with disease activity in juvenile dermatomyositis. , 1993, Archives of disease in childhood.

[20]  I. Lundberg,et al.  A clinical, serological, and histopathological study of myositis patients with and without anti-RNP antibodies. , 1992, Seminars in arthritis and rheumatism.

[21]  J. Schmidli,et al.  Quantitative nailfold capillary microscopy in cutaneous and systemic lupus erythematosus and localized and systemic scleroderma. , 1991, Journal of the American Academy of Dermatology.

[22]  H. Maricq,et al.  Childhood dermatomyositis: serial microvascular studies. , 1989, Pediatrics.

[23]  E. Keystone,et al.  Digital blood flow and nailfold capillary microscopy in Raynaud's phenomenon. , 1986, The Journal of rheumatology.

[24]  H. Maricq,et al.  Serial changes in nailfold capillary morphology in childhood dermatomyositis. , 1983, Arthritis and rheumatism.

[25]  W. Crowe,et al.  Nailfold capillary abnormalities in childhood rheumatic diseases. , 1983, The Journal of pediatrics.

[26]  W. Crowe,et al.  Nailfold capillary abnormalities and clinical outcome in childhood dermatomyositis. , 1982, Arthritis and rheumatism.

[27]  H. Maricq,et al.  Blind evaluation of the diagnostic specificity of nailfold capillary microscopy in the connective tissue diseases. , 1981, Arthritis and rheumatism.

[28]  A. Bohan,et al.  Polymyositis and dermatomyositis (second of two parts). , 1975 .

[29]  H. Maricq,et al.  Patterns of finger capillary abnormalities in connective tissue disease by "wide-field" microscopy. , 1973, Arthritis and rheumatism.