To describe a case of a young woman diagnosed with Sneddon’s Syndrome (SS) through a comprehensive and interdisciplinary evaluation.

A 25-year-old woman recently immigrated from the Dominican Republic with history of systemic lupus erythematosus (SLE) diagnosed at age 8 after presenting with malar rash, proteinuria, low complement, and thrombocytopenia with anti-nuclear antibody positivity currently on hydroxychloroquine, epilepsy diagnosed at age 8, endocarditis at age 13, ischemic stroke at age 19, hypertension, and miscarriage who was admitted to our hospital with a seizure.

Case Report

On exam, she had livedo racemosa and no neurological deficits. MRI showed punctate restricted diffusion in the left caudate body and extensive periventricular subcortical and white matter FLAIR hyperintensities. Transthoracic echocardiography and laboratory tests demonstrated Libman-Sacks endocarditis and lupus anticoagulant (positive twice 3 months apart). Skin biopsy showed vasculitis or thrombotic vasculopathy. She did not meet criteria for active SLE. She was initiated on warfarin, maintained on hydroxychloroquine, and discharged.

SS is a rare (4 per million annual incidence) medium-vessel vasculopathy characterized by cerebrovascular disease and livedo racemosa. The pathophysiology of SS is incompletely characterized, although antiphospholipid syndrome (APLS) and SLE are associated. The diagnosis is clinical, aided by thorough dermatologic examination, and can be delayed or missed due to the vast constellation of symptoms and unfamiliarity with the disease. This case report is the first to identify Sneddon’s syndrome in a patient with remote SLE and secondary APLS and identifies possible increased risk of SS in patients with SLE and APLS.