Case report of anti-transcription intermediary factor-1-γ/α antibody-positive dermatomyositis associated with gastric cancer and immunoglobulin G4-positive pulmonary inflammatory pseudotumor.

Abstract

Dermatomyositis is a rare connective tissue disease often associated with internal malignancy and interstitial pneumonitis. Serologically, various auto-antibodies (Ab) are associated with dermatomyositis. Anti-transcription intermediary factor-1-γ/α (TIF-1-γ/α) Ab was recently identified as an auto-Ab and was observed mostly in cancer-associated dermatomyositis. IgG4-related disease is a newly described entity characterized by increased serum IgG4 levels and IgG4-positive plasma cell infiltration with fibrosis in organs such as the pancreas and parotid gland. IgG4-related disease also includes inflammatory pseudotumors in various organs. We report herein a 59-year-old Japanese man who had dermatomyositis complicated with a gastric cancer and an IgG4-related pulmonary inflammatory pseudotumor. He manifested typical classical Gottron's papules on the fingers, V-sign erythema on the chest, flagellate erythema on the back, nail fold bleeding and facial erythema. Serum levels of anti-TIF-1-γ/α Ab were positive as assessed by immunoprecipitation assay. He also had bilateral swelling of the parotid gland, and an excised specimen of the lung showed inflammatory pseudotumor with IgG4-positive plasma cells. As far as we know, this case is the first to report the association of IgG4-related disease and TIF-1-γ/α-positive dermatomyositis. Further accumulation of such cases is required to elucidate the mechanism of this association.

• Authors: Kotaro Ito, Shinichi Imafuku, Yasuhito Hamaguchi, Manabu Fujimoto, Juichiro Nakayama
• Journal: The Journal of dermatology (J Dermatol) Vol. 40 Issue 7 Pg. 567-9 (Jul 2013) ISSN: 1346-8138 [Electronic] England
• PMID: 23663102 (Publication Type: Case Reports, Journal Article)
• Copyright: © 2013 Japanese Dermatological Association.

Chemical References

• Immunoglobulin G
• TRIM33 protein, human
• Transcription Factors

Topics

• Adenocarcinoma (complications, immunology)
• Dermatomyositis (immunology)
• Humans
• Immunoglobulin G (blood)
• Male
• Middle Aged
• Paraneoplastic Syndromes (immunology)
• Plasma Cell Granuloma, Pulmonary (complications, immunology)
• Stomach Neoplasms (complications, immunology)
• Transcription Factors (blood)