To the Editor:
Antineutrophil cytoplasmic antibody (ANCA) positivity in the setting of asymptomatic systemic sclerosis (SSc) does not consistently correlate with clinical characteristics. We describe a 31-year-old Latina woman with a 10-year history of limited SSc [sclerodactyly, tight facial skin, antinuclear antibody (ANA)-positive, gastroesophageal reflux disease, Raynaud’s phenomenon with no previous digital ulcerations, and without sicca-related symptoms or pulmonary or cardiovascular disease], who presented with severe digital necrosis. Her SSc had been stable under therapy with lansoprazole, hydroxychloroquine, celecoxib, and nifedipine.
Three months before admission, she developed muscle and joint pain in her distal extremities, with temporary relief taking oral and intravenous methylprednisolone. However, the purple discoloration and painful swelling of her toes progressed, and was unresponsive to lidocaine sympathectomies. Subsequently, she developed leg edema and rash, with numbness, tingling, and blistering. Three days before admission, the tips of her fingers and toes turned black.
Upon admission, her vital signs were stable. Examinations of head, ears, eyes, nose, throat, heart, and lung were normal. There was bilateral edema, cyanosis, and severe tenderness of distal extremities, without synovitis. There was diffuse superficial, livedo-like purpura on her thighs, frank necrosis of the tips of all her toes and 4 fingers, but no ulcerative lesions (Figures 1A, 1B). Her upper …
Address correspondence to Dr. Wong; E-mail: maidawong{at}mednet.ucla.edu