Referral for Livedoid Vasculopathy Bloodwork
For patients with livedoid vasculopathy, refer to hematology or a specialized coagulation clinic for comprehensive thrombophilia workup, not rheumatology, as this is a thrombotic disorder of dermal microcirculation rather than an inflammatory vasculitis.
Why Hematology, Not Rheumatology
Livedoid vasculopathy is fundamentally a coagulation disorder characterized by thrombotic occlusion of dermal vessels, not an inflammatory vasculitis despite its misleading name 1, 2. The condition results from hypercoagulable states and requires evaluation by specialists in hemostasis and thrombosis 3.
While rheumatology may be involved if there are associated autoimmune connective tissue diseases, the primary pathophysiology is thrombotic, making hematology the appropriate first referral for bloodwork 1.
Essential Bloodwork to Order
The hematology consultation should include comprehensive thrombophilia screening:
Inherited Thrombophilias
- Factor V Leiden mutation (activated protein C resistance) - this specific mutation has been documented in livedoid vasculopathy cases and should be tested via PCR analysis 4
- Prothrombin G20210A mutation 3
- Protein C, Protein S, and Antithrombin deficiencies 2
Acquired Thrombophilias
- Antiphospholipid antibodies (lupus anticoagulant, anticardiolipin antibodies, anti-β2-glycoprotein I antibodies) 2
- Homocysteine levels - frequently elevated in livedoid vasculopathy patients (10/12 patients in one cohort) 3
- Lipoprotein(a) - increased in approximately 42% of patients 3
Additional Screening
- Cryoglobulins and circulating immune complexes 4
- Antinuclear antibodies (ANA) and c-ANCA - to screen for associated autoimmune conditions, though typically negative or low-titer in primary livedoid vasculopathy 4
- Complete blood count and coagulation studies (PT/INR, aPTT) 2
When Rheumatology Referral Is Appropriate
Consider rheumatology consultation only if screening reveals:
- Positive autoimmune markers suggesting underlying connective tissue disease 1
- Clinical features of systemic autoimmune disease beyond the skin findings 2
However, this should be secondary to hematology evaluation, as the primary therapeutic approach involves anticoagulation, not immunosuppression 3.
Common Pitfall to Avoid
Do not treat livedoid vasculopathy with anti-inflammatory or immunosuppressive regimens first-line. Despite being frequently prescribed (17/24 patients in one study), these therapies showed zero effectiveness (0/17 responders), while anticoagulants like heparin demonstrated the highest efficacy (12/17 responders) 3. The German S1 guideline recommends anticoagulation with low-molecular-weight heparins, rivaroxaban, or other direct oral anticoagulants as first-line therapy 5.
Additional Cardiovascular Risk Assessment
Patients with livedoid vasculopathy frequently have cardiovascular risk factors that should be evaluated:
- Body Mass Index - elevated in approximately 41% of patients 3
- Hypertension - present in 70% of patients 3
- Lipid panel - given the association with elevated lipoprotein(a) 3
These assessments can be coordinated through hematology or your primary care, but are important for comprehensive management of the underlying hypercoagulable state 3.