Livedoid Vasculopathy Workup
- Author: Noah S Scheinfeld, JD, MD, FAAD; Chief Editor: William D James, MD more...
No specific laboratory examinations allow the physician to make a definitive diagnosis of livedoid vasculopathy, although levels of platelet P-selectin and endothelial thrombomodulin are elevated. Tests that assess the causes of diseases that result in lower leg ulcers can be used to diagnose other diseases but not livedoid vasculopathy.
Anavekar and Kelly noted a heterozygous prothrombin gene mutation associated with livedoid vasculopathy.
Evaluation of a patient can include appropriate imaging studies to evaluate for venous and arterial peripheral vascular disease. For example, venous Doppler studies can be useful in evaluating the disease. Microcirculation can be studied by capillary microscopy, transcutaneous oxygen measurements, laser Doppler flowmetry, laser Doppler perfusion imaging, and microlymphography.
A skin biopsy can be used to evaluate this condition.
The findings of livedoid vasculopathy are summarized in Histologic Diagnosis of InflammatorySkin Diseases:An Algorithmic Method Based on Pattern Analysis by Ackerman et al.
Histopathologic findings in the early stage include the following:
Sparse perivascular infiltrate of lymphocytes
Fibrin within the walls and fibrin thrombi within the lumen of venules in the upper part of the dermis (most cases)
Involvement of the lower half of the dermis (sometimes)
Histopathologic findings at the full stage of disease include the following:
Moderately dense, superficial and deep perivascular infiltrate of lymphocytes
Sparse neutrophils in the upper dermis
Fibrin in the walls of venules, in particular in the upper dermis
Thrombi occluding the lumen of venules in the upper dermis
Fibrin in the wall and thrombi in the lumen of the same venules in one or more venules
Large numbers of extravasated red blood cells in the upper part of the dermis
Edema of the papillary dermis
Spongiosis and ballooning sometimes resulting in intraepidermal vesiculation
Epidermal necrosis (sometimes)
Histopathologic findings in the late stage in which lesions of livedoid vasculopathy appear include the following:
Sparse infiltrate of lymphocytes mostly in the upper part of the dermis
Sclerosis in the upper part of the dermis
Numerous telangiectases in the upper part of the dermis
Epidermis thinned markedly and largely lacking rete ridges
Direct immunofluorescence staining typically demonstrates immunoglobulin and complement components in the superficial, mid-dermal, and deep dermal vasculature. The granular immunofluorescence staining pattern, typical of immune complex disease, was not seen prior to 2014. In 2014, Criado et al noted that the most common finding was a granular pattern of immunoreactant deposition, mainly C3 and immunoglobulin M, in a study of 36 patients of which 20 had laboratory pathologic findings and evidence of procoagulant findings (eg, anticardiolipin antibodies [37%], heterozygous factor V Leiden gene mutation [16%], protein C and/or S deficiency [16%], hyperhomocysteinemia [5%], lupus anticoagulant [25%], antithrombin deficiency [2.5%], high levels of fibrinogen [12%] and antinuclear factor [>1:320, 7.5%]).
Electron microscopy shows dilatation of capillaries (with a diameter up to 100 μm), with a thin endothelium, together with obliterated capillaries. Vessels are present in a dense, fibrotic connective tissue. Fibrin deposition with occlusion of the lumina of superficial blood vessels can occur. Erythrocytes and platelets are noted as being trapped within the fibrin. In older lesions, endothelial cells are replaced by heavy fibrin depositions.
Pathologic features vary with the stage of evolution of livedoid vasculopathy. In the early purpuric stage, fibrinoid material may be observed in the upper dermis in the capillary walls and the lumina. Endothelial proliferation and thickened walls are also noted. Deeper dermal and subcutaneous vessels are not involved in this stage.
In the late scarring phase of livedoid vasculopathy, which appears as atrophie blanche, the epidermis is thinned and the fibrinoid material has replaced the dermal vessels. Little or no cellular infiltrate is present. The pattern of involvement may vary; in some patients, the upper dermis is more involved than the deeper dermis, and, in other cases, the deeper layers show more of the changes mentioned above.
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