Dermatomyositis associated with hearing loss, peripheral nerve vasculitis and multi-system dysfunction


Classic myopathological features of dermatomyositis include perifascicular muscle fiber atrophy and perimysial perivascular inflammation, typically associated with focal microvascular depletion and membrane attack complex deposits on capillaries. The etiology behind microvascular injury however remains poorly understood. Co-existing systemic vasculitis is uncommon and peripheral nerve involvement has been rarely reported. A 39-year-old female, on Rituximab for “seronegative rheumatoid arthritis”, developed myalgias, night sweats, 30-pound weight loss and amenorrhea, followed by rapidly progressive proximal weakness.


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