Idiopathic livedo racemosa presenting with splenomegaly and diffuse lymphadenopathy

Sneddon syndrome (SS) is a rare condition and the diagnosis is made only when other more common disease entities have been excluded. Common manifestations in SS patients include hypertension, coronary artery disease, venous thrombosis, miscarriages, psychiatric disturbances, and arterial and venous thrombotic events. Most patients present in their early 30s with classic neurovascular and dermatologic signs. Currently, the main criteria for the diagnosis of SS include livedo racemosa, focal neurological deficits or evidence of stroke on magnetic resonance imaging, or characteristic vascular alterations seen on biopsy. We present the case of a 37-year-old woman with extensive livedo racemosa, chronic migraine headaches, splenomegaly, and lymphadenopathy. Cutaneous biopsies demonstrated a superficial perivascular lymphocytic infiltrate without the subendothelial proliferative changes or fibrosis seen in some patients with SS. The patient's medical history suggested idiopathic livedo racemosa with possible full progression to SS. This case highlights the variability in the clinical presentation of SS and that the disease often can be diagnosed before neurovascular events. Earlier diagnosis can lead to prevention of chronic occlusive neurovascular manifestations and irreversible damage such as myocardial infarction and stroke. Familiarity with the highly variable early course of SS can aid in diagnosis and reduction of morbidity and mortality that is associated with this disease.