2019 Research Forum

Applicant: Maryam Talai-Shahir MD R3 Principal Investigator & Investigator Sponsor: Arash Heidari MD

Heliotrope Rash Preceding Metastatic Ovarian Cancer

Maryam Talai-Shahir MD R3, Arash Heidari MD, Thy Nguyen MS IV

INTRODUCTION Dermatomyositis is a chronic inflammatory disease of the muscle and skin. The cardinal symptom is a heliotrope rash preceding progressive muscle weakness. The disease is rare, incidence of 0.5–0.89 per 100,000, female to male predominance 2:1. Recognizing it is challenging but important, as there appears to be an association with underlying malignancy as a paraneoplastic phenomenon. CASE PRESENTATION A 61-year-old Cambodian female presented to the emergency department with subjective fever and sharp, non-radiating right eye pain for three days. They were not associated with neuropathies or muscle pain. On examination, this patient was afebrile and was hemodynamically stable. A circumscribed raised plaquoid lesion was noted on the medial aspect of the right medial eye. There was erythematous excoriation of the right eyelid consistent with heliotrope rash. Musculoskeletal and neurological examination were normal. Initial lab work ruled out infectious, immunological, oncological etiologies. CT chest/abdomen/pelvis with contrast and colonoscopy showed no underlying malignancy. She was prescribed steroid cream, which helped improve the rash. However she was lost to follow-up until two years later when she presented to the emergency department with complain of severe diffuse abdominal pain, intractable nausea/vomiting, and heavy post-menopausal vaginal bleeding. Repeat CT chest/abdomen/pelvis with contrast showed a 10cm left ovarian mass. Lab finding was significant for elevated CA 125 of 1661U/ml. She subsequently underwent surgical resection of this tumor. She was diagnosed with stage IV ovarian carcinoma and stage IB1 squamous cell carcinoma of the cervix and chemotherapy was initiated. DISCUSSION Dermatomyositis as a paraneoplastic phenomenon in ovarian cancer has been reported in case reports. The diagnosis should be highly suspected based on clinical findings and inconclusive immunological findings. The interval from recognition of Dermatomyositis to development of underlying malignancy is variable. It can often be at the time of diagnosis or shortly thereafter. However, cases like our patient presentation when the underlying malignancy was not present at time of initial paraneoplastic phenomenon makes it difficult to assess appropriate follow-up if any. CONCLUSION A literature review does not reveal a timeline for follow-up or further investigation for underling malignancy. Most reported cases discuss the importance of evaluation for underlying malignancy at the time of presentation with Dermatomyositis. Even though this phenomenon is rare, its association with underlying malignancy is well known. Thus, patients should receive initial malignancy evaluation and yearly surveillance for malignancy screening to aid in early detection and management.

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