Paraneoplastic dermatomyositis with atypical features associated with a solid pseudopapillary pancreatic neoplasm

Area of Science:

• Autoimmune diseases
• Oncology
• Dermatology

Background:

• Dermatomyositis (DM) is an autoimmune inflammatory myopathy often associated with malignancies.
• Solid pseudopapillary pancreatic tumors are rare neoplasms with low malignant potential.
• The association between DM and solid pseudopapillary pancreatic tumors is exceedingly rare.

Purpose of the Study:

• To report an extremely rare case of dermatomyositis associated with a solid pseudopapillary pancreatic tumor.
• To highlight atypical cutaneous manifestations and anti-TIF1γ antibodies as potential indicators of paraneoplastic DM.
• To emphasize the importance of investigating rare pancreatic tumors in patients with DM and specific autoantibodies.

Main Methods:

• Case presentation of a 21-year-old woman with DM symptoms.
• Clinical evaluation including characteristic and atypical cutaneous lesions.
• Laboratory tests for muscle enzymes and anti-TIF1γ antibodies.
• Imaging studies to detect pancreatic mass and histological confirmation.
• Assessment of clinical response following surgical tumor resection.

Main Results:

• The patient presented with proximal muscle weakness, dysphagia, and characteristic DM skin lesions, plus atypical features like pseudoangioedema.
• Elevated muscle enzymes and positive anti-TIF1γ antibodies were observed.
• A solid pseudopapillary pancreatic tumor was diagnosed and surgically removed.
• Post-resection, muscle enzymes normalized, and skin lesions improved, though quadriparesis persisted.

Conclusions:

• Atypical cutaneous findings can indicate paraneoplastic dermatomyositis.
• The association of DM with solid pseudopapillary pancreatic tumors is exceptionally rare.
• This case underscores the need to consider rare pancreatic tumors in DM patients with anti-TIF1γ antibodies.
• Clinical improvement post-tumor resection supports a paraneoplastic link between the neoplasm and DM.