15397 Paraneoplastic dermatomyositis in a patient with an oligodendroglioma

Abstract

A previously healthy 26-year-old white man presented with nausea, headache, and rash. Physical examination disclosed coalescing, erythematous to violaceous macules and patches on the eyelids, periorbital regions, cheeks, upper chest, shoulders, upper back, and posterior neck, and mild periorbital edema. Review of systems was positive for myalgias. Histopathologic examination of a shoulder lesion demonstrated vacuolar interface dermatitis and a sparse, superficial perivascular dermal lymphocytic infiltrate. Colloidal iron staining demonstrated dermal mucinosis. Based on these findings, the diagnosis of dermatomyositis was made. Labs demonstrated elevated serum creatine kinase (673 U/L); tests for rheumatoid factor, anti-nuclear antibodies, anti-SSA and anti-SSB antibodies, immunoglobulin subclasses, and a myomarker panel were within normal limits. A head MRI demonstrated a cystic mass in the right insula; stereotactic biopsy was consistent with an oligodendroglioma. He received a 60 mg oral dose of prednisone, and topical triamcinolone 0.1% and hydrocortisone 2.5% ointment. Within 10 days the rash completely resolved. He underwent a craniotomy for tumor resection several weeks after initial presentation. At follow-up four months later, he showed no recurrence of myalgias or rash. Ovarian, lung, and gastrointestinal cancers are most commonly associated with dermatomyositis in the Western world. To our knowledge, only 2 previous cases of dermatomyositis associated with an intracranial neoplasm have been reported: in a 39-year-old man following dendritic cell immunotherapy for an oligoastrocytoma and in a seven-year-old girl with a choroid plexus papilloma. Our case adds oligodendroglioma to the list of potential neoplasms to be considered when assessing a patient with dermatomyositis.