15383 Dermatomyositis associated with ovarian cancer, negative screening, and negative antibodies

Abstract

A 60-year-old Caucasian female patient presented for a second opinion regarding a 9-month history of focal erythema on both dorsal hands. Her outside laboratory evaluation, including ANA, had a slightly elevated AST (40). No muscle enzyme studies had been performed. The “non-specific connective tissue disease” skin pathology report and the laboratory findings resulted in an initial diagnosis of “mixed connective tissue disease.” The patient was prescribed plaquenil but no improvement eventuated. The UTHealth McGovern Dermatology evaluation noted erythema of the scalp, forehead, eyelids, chest, dorsal forearms, dorsal hands, with erythema overlying the small joints of the hands with sparing of the tendon spaces. Muscle strength in upper and lower extremities remained intact. An 11-antibody dermatomyositis panel (including TIF1-γ) was negative. Creatine kinase and aldolase were elevated. Recent malignancy screening including CA-125, transvaginal ultrasound, mammogram, breast ultrasound, and colonoscopy were normal. CT of the chest, abdomen and pelvis was also normal. Once the patient proceeded with a previously scheduled elective TAH-BSO, a high-grade serous carcinoma involving bilateral ovaries and the right fallopian tube was discovered. The surgery and chemotherapy have resulted in marked improvement in the patient’s skin alterations and her overall sense of well-being. The transcriptional intermediary factor gamma (TIF1-γ) antibody is associated with malignancy in dermatomyositis and has a sensitivity of 78% and specificity of 89% for cancer but was negative in our patient. Aggressive workup and management may be warranted in patients with a high suspicion for disease despite negative screening and negative antibodies.