Correct!
1. All of the above
Infection can cause retiform purpura particularly meningococcemia which is a common cause of DIC. Her blood cultures showed 2 of 2 bottles cultured Gram positive cocci in pairs and chains.
Septic vasculitis presents with retiform purpura with meningococcemia, pseudomonal or streptococcal septicemia, gonococcemia, and rickettsial infections.
Purpura fulminans has a high mortality and high long-term morbidity. Gangrene in the acral areas is common and can require amputation of multiple digits after the patient stabilizes often late in the hospital course.
Upon presentation, dermatology, rheumatology, nephrology and infectious disease physicians were consulted and the consensus was that the extensive skin lesions were from severe DIC or purpura fulminans.
Several additional tests should have been performed to further evaluate this patient with recurrent pneumococcal bacteremia including determining whether she has a spleen; measuring her antibody levels and doing an HIV test as well. Patients with lupus can sometimes autoinfarct their spleen essentially creating a splenectomized patient.
Important questions arise given her history and presentation including the differential diagnosis from a rheumatologic standpoint, and does she need high dose steroids for disease flare, does she need urgent plasma exchange in the setting of life-threatening clinical condition with underlying SLE, and/or does she need IVIG related to ITP from lupus or cutaneous lupus?
Based on the initial presentation, what is the patient’s most likely rheumatological diagnosis? (Click on the correct answer to be directed to the fifth of six pages)