Correct!
5. None of the above
Discoid lupus causes round, coin-shaped lesions (sores). The sores most commonly develop on the scalp and face, but they may show up on other parts of your body. Discoid lesions typically do not hurt or itch. They may be scaly, thick or red. When the lesions go away, they may leave scars or skin discoloration.
Not known initially on admission the patient's double stranded DNA (DsDNA) levels which correlate with lupus disease flare were trending down even on this admission-321 on 10/6, 40 on 12/17, 18 on 12/20. C3 was slightly lower than previous value but sepsis itself can cause C3 to be lower. C4 level is similar to past levels. The main point regarding active lupus is to check DsDNA, C3 and C4 levels in order to trend values and help determine diagnosis.
Leukopenia often correlates with disease activity in lupus. Leukocytosis in lupus patients is worrisome for infection. Direct antiglobulin (Coombs) test was negative and severe thrombocytopenia is uncommon in lupus. Our patient’s labs are more suggestive of DIC.
Catastrophic antiphospholipid syndrome (CAPS) consists of widespread thrombotic disease with multiorgan failure. Classification criteria is 3 or more organs involved simultaneously accompanied by small vessel thrombosis in a patient with antiphospholipid antibodies. Testing involves IgG and IgM antibodies to cardiolipin, beta2-glycoprotein I and lupus anticoagulant (LA).
In this patient, there was no clear evidence of thrombosis, and aPL antibodies were negative in the past. The history of PE, left PICA territory infarct with venous thrombosis of left sigmoid sinus did make CAPS a strong possibility however.
Hemophagocytic lymphohistiocytosis (HLH) or macrophage activation syndrome (MAS) is an aggressive and life-threatening syndrome of excessive immune activation and can occur with rheumatologic disorders. The term macrophage activation syndrome (MAS) is used when HLH develops in a rheumatologic condition. In addition, HLH may develop any time during the course of a rheumatologic disorder. HLH presents with fever, skin rash/purpura, bicytopenia, splenomegaly, elevated liver function tests, hypertriglyceridemia, elevated ferritin, evidence of hemophagocytosis, NK cell activity, and elevated sCD25. In this patient's presentation, the leukocytosis goes against HLH/MAS, ferritin was 1403 (less than 3000), and slightly elevated sCD25 likely represent bacterial or viral infections.
If suspecting purpura fulminans, what lab tests should be ordered? (Click on the correct answer to be directed to the sixth and final page)
1. DIC panel
2. Protein C
3. Protein S
4. Peripheral smear
5. All of the above