Case Report: Dermatomyositis and Malignancy

High-grade müllerian carcinoma in a 75-year-old female

A 75-year-old female originally diagnosed with dermatomyositis in October 2020 presented in August 2021 with severe proximal weakness and dysphagia requiring a nasogastric tube placement. Creatine kinase and erythrocyte sedimentation rate were normal, and her C-reactive protein was slightly elevated. Electromyography suggested an inflammatory myopathy.

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Computed tomography of the chest, abdomen and pelvis indicated retroperitoneal lymphadenopathy, pulmonary ground-glass opacities and findings suggestive of pulmonary arterial hypertension. Retroperitoneal biopsy revealed a high-grade müllerian carcinoma. Cleveland Clinic Cancer Center specialists consulted with the patient regarding treatment options, but she decided to pursue hospice care and died two days later.

Dermatomyositis is associated with a six-fold higher risk of malignancy compared with the general population, especially within the first two years after diagnosis. Rheumatologists must remain vigilant in evaluating these patients for malignancy, especially when disease is refractory. 

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Dr. Yedimenko is staff in the Department of Rheumatologic and Immunologic Diseases.