Livedo Reticularis: Hereditary Nature and Cold-Induced Variant
Livedo reticularis is not typically hereditary, and when it occurs specifically in response to cold exposure, it is called physiological livedo reticularis (or cutis marmorata).
Primary vs Secondary Forms
Livedo reticularis presents as a violaceous, net-like mottling pattern on the skin and exists in both primary (benign) and secondary (pathologic) forms 1, 2.
Primary Livedo Reticularis
- Primary livedo reticularis is not hereditary and represents a benign vasospastic condition affecting mainly middle-aged females 3.
- The physiological variant appears transiently with cold exposure and resolves with rewarming 2, 3.
- This benign form is the most common type and requires no treatment in most cases 1.
Secondary Livedo Reticularis
- Secondary forms are associated with systemic diseases rather than genetic inheritance 1, 3.
- The pathologic variant, termed livedo racemosa, presents as an irregular, persistent pattern that does not resolve with warming 3, 4.
- Livedo racemosa is commonly associated with antiphospholipid antibody syndrome, which can present with venous and arterial occlusive disease in multiple organs, miscarriages, and livedo reticularis 5.
Cold-Induced Livedo: Terminology and Characteristics
When livedo reticularis occurs specifically in response to cold temperatures, it is termed "physiological livedo reticularis" 3, 4.
- This physiological variant is completely benign and related to normal vasospastic responses to cold 2.
- It appears transiently during cold exposure and disappears when the skin is warmed 3, 4.
- The pattern consists of regular, symmetric, net-like rings that blanch with pressure 2.
Clinical Distinction: Key Differentiating Features
Physiological (Cold-Induced) Pattern
- Appears only with cold exposure 3, 4
- Regular, symmetric fishnet pattern 1, 2
- Completely reversible with warming 3
- No associated systemic symptoms 1
Pathological Pattern (Livedo Racemosa)
- Persists in warm temperatures 4
- Irregular, broken, asymmetric pattern 3, 4
- Associated with systemic diseases 1, 3
- May indicate underlying thrombotic or inflammatory conditions 4
Management Approach
For Physiological Livedo Reticularis
- No treatment is required for asymptomatic patients with primary physiological livedo reticularis 1.
- If mild symptoms occur (episodic numbness, tingling), avoidance of cold and vasoconstricting substances is recommended 1.
- Calcium channel blockers can be prescribed judiciously for symptomatic cases 1.
For Secondary Forms
- The American Heart Association recommends antiphospholipid antibody testing for patients with suspected secondary livedo reticularis, particularly with systemic symptoms 6.
- Coagulation studies should be performed to evaluate for prothrombotic states 6.
- For antiphospholipid antibody syndrome with ischemic stroke or TIA, antiplatelet therapy with a target INR of 2-3 is reasonable 5.
- Critical caveat: The livedo pattern typically persists or progresses despite anticoagulation therapy, even when the underlying condition is adequately treated 1.
Common Pitfalls to Avoid
- Do not assume hereditary etiology: Livedo reticularis is acquired rather than inherited in the vast majority of cases 1, 3.
- Do not overlook systemic evaluation: Persistent livedo that does not resolve with warming requires investigation for secondary causes including antiphospholipid antibody syndrome, vasculitis, and thrombotic disorders 6, 3.
- Do not expect cutaneous resolution with anticoagulation: Even with appropriate treatment of underlying prothrombotic conditions, the livedo pattern often remains unchanged 1.