Rescue Treatment with Infliximab for a Bilateral, Severe, Sight Threatening Frosted Branch Angiitis Associated with Concomitant Acute Onset of Presumed Dermatomyositis.

Purpose: To report a severe bilateral frosted branch angiitis associated with acute onset of systemic dermatomyositis.

Method: History and clinical examination, laboratory evaluation, fundus' and skin's color pictures, fluorescein angiography (FA), optical coherence tomography (OCT).

Results: A 32-year-old female was referred for a bilateral frosted branch angiitis and vitreitis, with skin rash, muscle pain, weakness, severe headache, compatible with dermatomyositis. After an initial improvement following an aggressive treatment by systemic steroids and mycophenolate mofetil, she was switched to intravenous infliximab (RemicadeTM, Janssen Biotech, Inc. USA) due to lack of efficacy. Nine months later, her visual acuity was improved up to 20/20 in OU, with both retinal vasculitis and vitreitis being solved.

Conclusion: Dermatomyositis is a severe multiorgan disease which might severely involve eye structures. Anti-TNF-Alpha agents, particularly Infliximab, might offer a rapid control and long-term remission.