Severe Raynaud's phenomenon Leading to digital amputation in a patient with systemic lupus erythematosus.

Abstract

A 15-year-old female presented with low grade fever for one month. She developed hyperpigmented rash on her face for 2 weeks. She went to the hospital due to fever and drowsiness. Her vital signs showed hypotension and fever. She was treated as septic shock. She received antibiotic, intravenous fluid loading and vasopressor drug. Her physical examination showed hyperpigmented rash at face, sparing the nasolabial folds with maculopapular rash all over her body, no hepatosplenomegaly. Her laboratories showed anemia, leukopenia, transaminitis, high ferritin, ANA 1:160 coarse speckled with nRNP/Sm, anti Sm and anti SSA positive, anti-dsDNA negative, low C3, low C4, APL negative, Echocardiogram showed pericardial effusion 4 mm, clear fluid, EF 55%, no vegetation. Her bone marrow aspiration showed hemophagocytosis. She developed severe Raynaud’s phenomenon despite intensive vasodilator and immunosuppressive therapy. Progressive digital gangrene eventually required amputation of all her fingers on both hands.

        In summary, Raynaud’s phenomenon can progress to digital gangrene, particularly in the presence of additional factors such as vasopressor use. Early recognition and prevention are essential.