Ocular multimodal imaging of a patient with malignant atrophic papulosis (Degos disease)

Purpose: To report multimodal imaging of a patient with Degos disease. Results: A 37-year-old male presented with typical Degos disease skin lesions, blurred near vision, peri-central right scotoma along with right mid-dilated unreactive pupil, no afferent pupillary defect, left ptosis and exotropia. He developed right painless conjunctival ischemia with dilated perilesional vessels and was treated by eculizumab and prostacyclin analogs. Despite four months of treatment, chorioretinal ischemia with subsequent atrophy appeared. Worsening of extracutaneous symptoms led to a treatment change (increased eculizumab dosage, addition of baracitinib and anifrolumab). Consequently, pupils, ptosis and exotropia fully resolved, however the conjunctiva exhibited incomplete vascularization and the retina displayed sequelae consistent with chorioretinal infarction. Conclusions: Degos disease is a lethal vasculopathy that can involve several eye structures. It seems to have a peculiar tropism for conjunctival and choroidal vessels with severe infarctions. Lesions can be reversible if promptly managed in a tertiary specialized center.