The clinical presentation of
dermatomyositis (DM) is diverse, with varied phenotypes that may be correlated with specific
autoantibodies. The anti-
melanoma differentiation-associated gene 5 (MDA5) antibody in DM is associated with an amyopathic phenotype of DM, with several unusual cutaneous manifestation and increased risk for rapidly progressive
interstitial lung disease. Initial presentation may be subtle, but early diagnosis is key to initiation of proper immunosuppressive therapy. In this report, we describe perinasal
edema and
erythema as a presenting complaint of anti-MDA5 DM in an otherwise healthy 40-year-old woman. The
edema began shortly after heavy sun exposure and was followed by painful papules in her hands and
arthritis within a few weeks. She was found to have high titer of
anti-CCP and anti-MDA5, and thus was diagnosed with DM and
rheumatoid arthritis overlap. A CT chest, abdomen, and pelvis showed patchy ground-glass and interstitial opacities in bilateral lower lobes consistent with mild
interstitial lung disease without evidence of
malignancy. Perinasal cutaneous findings and
arthralgias improved with initiation of
prednisone. To our knowledge, this is the first report of perinasal
edema as a presenting symptom for DM and should raise suspicion for MDA-5 disease.