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Dermatomyositis (DM) classically presents as a dyad of typical cutaneous findings and varying degrees of proximal muscle weakness. Interestingly, the development of DM may signal underlying malignancy, and numerous myositis-specific autoantibodies have been associated with this paraneoplastic phenomenon. Positivity for anti-TIF1gamma antibody, in particular, raises suspicion for cancer-associated DM. Here, we present an unusual case of anti-TIF1gamma antibody-positive DM that ultimately lead to the diagnosis of myelodysplastic syndrome (MDS). Importantly, topical treatment and chemotherapy targeting MDS resulted in a swift and remarkable amelioration of cutaneous disease.

Anti-TIF1gamma Antibody-Positive Dermatomyositis Associated with Myelodysplastic Syndrome: Response to Treatment