A Case of Dematomyositis in a Cancer Patient Presenting as Dysphagia

Abstract

Dermatomyositis (DM) is a rare idiopathic inflammatory muscle and skin disorder with a 2:1 female predominance, and peak incidence in the 5th and 6th decades of life. DM presents acutely or progressively as painless proximal skeletal muscle weakness and can be associated with the characteristic heliotropic rash around the eyes and Gottron's papules on extensor surface of the hands. Individuals may also present with symptoms of dysphagia, and cardiac or pulmonary involvement. 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 difficulty swallowing over the past three months. She also reported difficulty ambulating, facial rash, and inability to extend her fingers. Physical examination revealed a cachectic-appearing woman and areas of hyperpigmentation on her face, arms, and legs. She also had symmetric proximal muscle weakness in both upper and lower extremities. Laboratory findings were significant for an elevated serum creatinine phosphokinase level of 3222 U/L, creatine kinase isoform MB 369.7 ng/mL, aldolase 31.7 U/L, erythrocyte sedimentation rate 40 mm/hr, C-reactive protein 8.41 mg/L, lactate dehydrogenase 804 U/L, and antinuclear antibody positive >1:640. Barium esophagram revealed significant difficulty initiating swallow with laryngeal penetration and aspiration of contrast. Esophagogastroduodenoscopy showed pooling of saliva in the piriform fossa with a normal appearing esophagus. A left biceps muscle biopsy revealed inflammatory myositis with significant necrotizing component suggestive of DM. Further staining with nicotinamide adenine dinucleotide tetrazolium reductase (NADH-TR) stain identified frequent myofibers with targetoid-like changes consistent with DM. She was treated with intravenous corticosteroids, however, she did not respond to the treatment. Thus, a percutaneous gastrostomy tube was placed for enteral feeding. DM is an inflammatory myopathy with cutaneous manifestations and associated with malignancy. Striated muscle in the oropharynx and upper one-third of the esophagus are affected, which results in oropharyngeal dysphagia. Patients with muscle weakness and progressive dysphagia can be a hallmark of malignancy. In our patient, DM presented as dysphagia with multiple malignancies with lack of a response to corticosteroid treatment, which portends a poor prognosis.Figure 1Figure 2Figure 3