Periorbital heliotrope erythema with edema as a potential clinical marker of paraneoplastic Dermatomyositis – a case series

Adult-onset dermatomyositis (DM) is frequently associated with underlying malignancies, especially in individuals with anti-TIF1-γ or anti-NXP-2 autoantibodies. This case series describes 10 cases of DM with periorbital heliotropic erythema and eyelid edema (PHEEE), in which an underlying malignancy was confirmed by imaging in 9 patients. By incorporating additional serological markers and clinical characteristics described in the literature, a simple criteria list is provided to aid in the assessment of a paraneoplastic etiology in DM. The presence of PHEE appears to constitute a clinically relevant indicator of paraneoplastic DM and requires careful screening for malignancy.