A 55 yo M with history of T2DM, HTN, and HLD presents with unintentional weight loss. He also has abdominal pain and a new rash.
Questions to Ask
- Other systemic symptoms?
- Duration of symptoms?
- Any other body systems involved?
- Any risk factors for infection or malignancy?
History & Physical Exam
- He is having subjective fever and night sweats.
- On exam, he is ill-appearing and febrile (38.4C).
- Exam shows his rash to be consistent with livedo racemosa, and there are sharply demarcated ulcerative lesions on the legs.
- Abdominal exam is relatively unremarkable, but he has R testicular tenderness.
Diagnostic Workup
- Initial workup:
- CBC w/diff, CMP, UA, ESR, CRP, ANCA, HIV and hepatitis serologies, blood cultures
- Echo
- Deep skin biopsy of an ulcerative lesion
- To consider if Dx still unclear:
- Invasive angiography
Further Workup & Referrals
- Full thickness biopsy of ulcer looking for medium vessel vasculitis
- Expedited referral to rheumatology for high-dose IV corticosteroids
Treatment & Management
- Treatment with high-dose steroids +/- cyclophosphamide depending on imaging findings and presence of organ threatening disease (under rheumatology care)
- Monitor glucose, blood pressure, blood counts, UA (hemorrhagic cystitis risk), evidence of infection, secondary malignancy
A 59 yo female with history of obesity and iron-deficiency anemia presents with transient monocular vision loss, as well as subacute fatigue and malaise.
Questions to Ask
- Any other eye symptoms?
- Duration of symptoms?
- Any other body systems involved?
- Traditional cardiovascular risk factors?
- Cardioembolic risk factors?
History & Physical Exam
- 2 episodes of acute R eye complete vision loss that lasted 3-5 min before resolving
- No current headache, diplopia, or jaw claudication
- Vision and external eye exams are normal.
- She also developed L wrist weakness suddenly 2 weeks ago, and both a L wrist extension and R dorsiflexion weakness are noted on exam.
- Cardiac, pulmonary, skin, HEENT, and joint exams are normal.
Diagnostic Workup
- Initial workup:
- CBC w/diff, CMP, ESR, CRP, ANCA, HIV, HBV, blood cultures
- Echo
- EMG
- Consider:
- Sural nerve biopsy
- Temporal artery biopsy
Further Workup & Next Steps
- This severe presentation warrants hospitalization and empiric therapy (steroids).
- Initial workup should prioritize evaluation for systemic inflammation and characterization of her neurologic deficits.
- GCA is the most likely vasculitis to cause amaurosis, but can happen with PAN
- If there is mononeuritis multiplex (foot drop, hand drop), PAN or ANCA-associated vasculitis would be more likely.
- Deep biopsy is helpful to identify if this is medium vessel vasculitis.