A RARE CASE OF PARANEOPLASTIC EDEMATOUS DERMATOMYOSITIS ASSOCIATED WITH SMALL CELL LUNG CANCER

Abstract

SESSION TITLE: Lung Cancer: Unusual Presentations SESSION TYPE: Med Student/Res Case Report PRESENTED ON: 10/21/2019 3:15 PM - 4:15 PM INTRODUCTION: Paraneoplastic edematous dermatomyositis (EDM) as a manifestation of Small Cell Lung Cancer (SCLC) is a rare process with one reported case in the literature. CASE PRESENTATION: We present a 51-year-old, previously healthy woman with a nine pack-year smoking history, who presented with a one-month history of progressive facial edema, facial erythema, lip swelling, and a violaceous atypical rash with crusting lesions involving her extremities, anterior neck, chest wall (V sign), and periungual area. The erythematous rash with blistering initially developed on the undersurface of both forearms after exposure to a wheelchair which the patient attributed to a nickel allergy. The patient failed low-dose outpatient steroids and was admitted with progressive lip swelling and facial edema. Over the course of her hospitalization, the patient developed significant proximal muscle weakness, proximal muscle tenderness, and was found to have an elevated creatine kinase level of 5,912 [IU]/L, antinuclear antibody titer was positive at 1:640 dilution, and a dermal biopsy consistent with dermatomyositis. Chest CT revealed a hilar mass with precarinal adenopathy. The patient underwent endobronchial ultrasound with transbronchial aspirate demonstrating SCLC. The patient’s facial edema and atypical rash responded to high-dose oral prednisone although proximal weakness persisted at discharge from the hospital. Upon follow up with hematology and oncology, the patient was formally diagnosed with limited stage SCLC and initiated on etoposide and carboplatin with radiation therapy. Additional evaluation with CT Abdomen demonstrated no evidence of distant metastasis. After completion of her chemoradiotherapy, her subcutaneous edematous rash resolved. DISCUSSION: A review of the literature suggests that EDM is rare with only 23 cases reported to date. EDM may be associated with a higher mortality and a more fulminant course. CONCLUSIONS: Familiarity with this paraneoplastic phenomenon may lead to early diagnosis of treatable malignancies and decrease short term mortality. Reference #1: Zarrabi, K., et al., Paraneoplastic edematous dermatomyositis: A rare syndromeobserved in a case of small cell lung cancer. Clinics and practice, 2017. 7(4). Reference #2: Milisenda JC, Doti PI, Prieto-González S, Grau JM. Dermatomyositis presenting withsevere subcutaneous edema: five additional cases and review of the literature. SeminArthritis Rheum 2014; 44:228. DISCLOSURES: No relevant relationships by James Davidson, source=Web Response No relevant relationships by Jeremy Harvey, source=Web Response No relevant relationships by Rahul Sampath, source=Web Response No relevant relationships by Mathew Thomas, source=Web Response No relevant relationships by Ly Tran, source=Web Response