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Updated: May 5, 2026
Imaging CD4 T Cell Interstitial Migration in the Inflamed Dermis
Published on: March 25, 2016
Recurrent anti-TIF1γ-positive dermatomyositis coexisting with postoperative parotid lymphoepithelial carcinoma: a
Xiaoguang Cui1,2, Kaihong Ye3, Hong Wang4
1National-Local Joint Engineering Research Center of Biodiagnosis & Biotherapy, The Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi, China.
Background:
Anti-TIF1γ-positive dermatomyositis (DM) is a classic paraneoplastic syndrome in adults, but its coexisting with lymphoepithelial carcinoma (LEC) of the parotid gland is exceptionally rare. This rarity poses significant challenges for clinical management.
Objective:
To report a unique case of parotid LEC emerging three years after a diagnosis of anti-TIF1γ-positive DM, followed by a post-oncologic DM recurrence. We aimed to investigate the underlying immunopathogenesis through peripheral blood mononuclear cell (PBMC) analysis and genetic profiling.
Case Presentation:
A 28-year-old male presented with anti-TIF1γ-positive DM. Three years later, he developed parotid LEC, with Epstein-Barr virus (EBV) detected in both tumor tissue and serology. He was treated with surgical resection and adjuvant therapy, achieving a near-complete oncologic response. However, DM recurred eight months after the cancer diagnosis. Initial cyclophosphamide treatment was effective, but its withdrawal led to relapse; subsequent therapies with methotrexate and tofacitinib provided minimal benefit.
Results:
PBMC analysis during the DM recurrence revealed a highly active B-cell population and a reduction in cytotoxic cells. This B-cell expansion subsequently decreased 10 months later, suggesting a delayed effect of the documented EBV activation. Germline genotyping identified a panel of deleterious germline mutations in immune regulation genes, including a variant in CR2 (rs367567954), which encodes a receptor for EBV on B cells and may contribute to their aberrant activation.
Conclusion:
This case illustrates that refractory anti-TIF1γ-DM can persist even after the associated malignancy is well controlled and underscore the need for long-term vigilance and personalized management strategies in paraneoplastic DM.
