Purtscher-like retinopathy associated with pemphigus vulgaris

Abstract

Purpose: To report a case of Purtscher-like retinopathy associated with pemphigus vulgaris.

Method: Case report of a 25-year-old Hispanic male who was referred for sudden and severe reduction of vision from his right eye shortly after an exacerbation of pemphigus vulgaris.

Results: Fundus examination of the right eye revealed severe macular edema, nerve fiber layer infarcts, flame-shaped hemorrhages, and venous dilation. Fluorescein angiography and optical coherence tomography findings of flecken, ischemia with subsequent nerve fiber layer atrophy, and capillary leakage were consistent with Purtscher-like retinopathy. The patient was treated with 5 consecutive monthly intravitreal injections of 0.5 mg of ranibizumab. Visual acuity improved with resolution of macular edema, and the patient was started on systemic mycophenolate mofetil. Continued ocular stability was achieved with 11 subsequent intravitreal injections over the following 2 years.

Conclusion: Purtscher-like retinopathy may have been associated with pemphigus vulgaris through a complement activation process that is previously undescribed. Retinal findings were successfully mitigated with the use of intravitreal anti-vascular endothelial growth factor therapy.