Why are my toes blue?

A case of a 40 year old woman from Honduras presenting with Raynaud’s disease to Rheumatology clinic – cyanosis and ulceration of her left foot digits. She had atypical features of primary Raynaud’s – age > 30, asymmetric digit cyanosis, sclerodactyly/hand skin thickening – which prompted further work-up.  She was found to have high titer ANA (1:1280) with anti-centromere pattern which further supports her diagnosis of limited scleroderma. She was also tested for hypercoagulable markers given her degree of and asymmetry of ischemia on presentation and found to have positive lupus anticoagulant, anti-cardiolipin antibodies, and B2-glycoprotein antibodies. She returned with worsening symptoms on the left foot despite therapy with nifedipine for vasospasm.  She was admitted to start on IV vasodilators (epoprostenol) and anti-coagulation (LMWH as bridge to coumadin) to treat both possible vasospasm and thrombosis — should she have imaging to diagnose a thrombosis so we don’t commit her to lifelong anticoagulation unnecessarily?  Be careful to watch for any signs of infection in the ischemic digits as they can be difficult to treat!

Some nice reviews:

Raynaud’s

Scleroderma

Anti-phospholipid syndrome

Digit ischemia in scleroderma