Distinctive cutaneous and systemic features associated with antitranscriptional intermediary factor-1γ antibodies in adults with dermatomyositis

David F. Fiorentino, Karen Kuo, Lorinda Chung, Lisa Zaba, Shufeng Li, Livia Casciola-Rosen

Research output: Contribution to journalArticlepeer-review

82 Scopus citations


Background Antibodies against transcriptional intermediary factor (TIF)-1γ are associated with malignancy in dermatomyositis (DM). Identification of clinical findings associated with anti-TIF-1γ antibodies in DM is a high priority for both patient diagnosis and risk assessment. Objective We sought to define the clinical phenotype of patients with anti-TIF-1γ DM. Methods Using a novel, sensitive, and specific assay for anti-TIF-1γ antibodies, we retrospectively tested plasma from 134 adult patients with DM and examined associations between anti-TIF-1γ antibodies and particular clinical and laboratory features. Results In all, 55 (41%) patients had autoantibodies to TIF-1γ. Anti-TIF-1γ positive patients were less likely to have systemic features including interstitial lung disease, Raynaud phenomenon, and arthritis/arthralgia. Patients with TIF-1γ autoantibodies had more extensive skin involvement, and some patients manifested characteristic findings including palmar hyperkeratotic papules, psoriasis-like lesions and a novel finding of hypopigmented and telangiectatic ("red on white") patches. Limitations This was a retrospective study from a single tertiary referral center. Conclusion TIF-1γ is the most commonly targeted DM-specific autoantigen in adults in a large US cohort. Although these patients tend to have less systemic involvement, their skin disease is often extensive and characteristic. Recognition of cutaneous findings in anti-TIF-1γ positive patients may allow more accurate and timely diagnosis and effective treatment of patients with DM.

Original languageEnglish (US)
Pages (from-to)449-455
Number of pages7
JournalJournal of the American Academy of Dermatology
Issue number3
StatePublished - Mar 1 2015


  • Cutaneous Dermatomyositis Assessment and Severity Index
  • autoantibodies
  • dermatomyositis
  • malignancy
  • phenotype
  • transcriptional intermediary factor-1γ

ASJC Scopus subject areas

  • Dermatology


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