Paraneoplastic Dermatomyositis Syndrome Presenting as Dysphagia

Abstract

Dermatomyositis (DM) is a rare autoimmune condition which predominantly affects females in the fifth and sixth decades of life. DM presents acutely or progressively as painless proximal skeletal muscle weakness and can be associated with a heliotropic rash around the eyes, and Gottrons papules on extensor surfaces of the hands. While the pathophysiology of DM is still unclear, abnormal T- and B-cell immune activity has been reported. DM has been associated with malignancy and has been shown to appear before, concurrently with, or after diagnosis of cancer. We report a 72-year-old female with renal clear cell carcinoma, breast cancer, and papillary serous carcinoma of the uterus, who presented with progressive weakness, 30 pound weight loss, and dysphagia over the past 3 months. She also reported difficulty in ambulating, facial rash, and inability to extend her fingers. Left bicep muscle biopsy and staining with nicotinamide adenine dinucleotide tetrazolium reductase (NADH-TR) identified frequent myofibers with targetoid-like changes consistent with DM. Despite immunosuppression with intravenous corticosteroids, she did not respond to the treatment and a percutaneous gastrostomy tube was placed for enteral feeding.




Gastroenterol Res. 2017;10(4):251-254
doi: https://doi.org/10.14740/gr841w