title: Cutaneous ulceration in dermatomyositis: association with anti-melanoma differentiation-associated gene 5 antibodies and interstitial lung disease.
abstract: OBJECTIVE
  To identify clinical and serologic correlates of cutaneous ulcers in dermatomyositis (DM).
  
  
  METHODS
  We retrospectively examined a cohort of 152 DM patients. We compared the features of patients with ulcers to those without ulcers using chi-square or Fisher's exact tests and used univariate and multivariate logistic regression models to assess the association between ulcers and clinical features such as malignancy, interstitial lung disease (ILD), and amyopathic disease.
  
  
  RESULTS
  Forty-three patients (28%) had cutaneous ulcers. Nearly half the patients had ulcers present in more than 1 location: 24 (56%) had ulcers over the extensor surfaces of joints, 18 (42%) at the digital pulp or periungual areas, and 25 (58%) had ulcers located elsewhere. In univariate analysis ulcers were associated with Asian race, but not with other clinical and demographic features, including malignancy or ILD. In multivariate analysis ulcers were significantly associated with anti-melanoma differentiation gene 5 (anti-MDA5) antibodies (odds ratio 10.14, 95% confidence interval 1.95-52.78; P = 0.0059) and this was greatest for ulcers located at the digital pulp. In patients with cutaneous ulcers, ILD risk was specifically increased only in patients with anti-MDA5-positive antibodies.
  
  
  CONCLUSIONS
  We confirmed the strong association between anti-MDA5 antibodies and cutaneous ulcers, with the novel finding that the association of cutaneous ulcers with ILD depends upon the presence of anti-MDA5 antibodies. DM patients who display this cutaneous phenotype should undergo appropriate evaluation for ILD.
authors.affiliation: Stanford University, Stanford, California.
authors.forename: Neera S
authors.initials: NS
authors.lastname: Narang
authors.forename: Livia
authors.initials: L
authors.lastname: Casciola-Rosen
authors.forename: Shufeng
authors.initials: S
authors.lastname: Li
authors.forename: Lorinda
authors.initials: L
authors.lastname: Chung
authors.forename: David F
authors.initials: DF
authors.lastname: Fiorentino
mesh_terms: D000328:Adult; D001213:Asian; D001323:Autoantibodies / Q000097:blood*; D002140:California / Q000453:epidemiology; D016009:Chi-Square Distribution; D053487:DEAD-box RNA Helicases / Q000276:immunology*; D003882:Dermatomyositis / Q000097:blood / Q000150:complications* / Q000175:diagnosis / Q000208:ethnology / Q000276:immunology; D005260:Female; D006801:Humans; D000072640:Interferon-Induced Helicase, IFIH1; D016015:Logistic Models; D017563:Lung Diseases, Interstitial / Q000097:blood / Q000175:diagnosis / Q000208:ethnology / Q000209:etiology* / Q000276:immunology; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D016017:Odds Ratio; D012189:Retrospective Studies; D012307:Risk Factors; D012883:Skin Ulcer / Q000097:blood / Q000175:diagnosis / Q000208:ethnology / Q000209:etiology* / Q000276:immunology
chemical_list: D001323:Autoantibodies; C452815:IFIH1 protein, human; D053487:DEAD-box RNA Helicases; D000072640:Interferon-Induced Helicase, IFIH1