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Livedoid Vasculopathy Workup

  • Author: Noah S Scheinfeld, JD, MD, FAAD; Chief Editor: William D James, MD  more...
 
Updated: Jan 25, 2016
 

Laboratory Studies

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[32] noted a heterozygous prothrombin gene mutation associated with livedoid vasculopathy.

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Imaging Studies

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.

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Procedures

A skin biopsy can be used to evaluate this condition.

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Histologic Findings

The findings of livedoid vasculopathy are summarized in Histologic Diagnosis of InflammatorySkin Diseases:An Algorithmic Method Based on Pattern Analysis by Ackerman et al.[33]

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[34] 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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Contributor Information and Disclosures
Author

Noah S Scheinfeld, JD, MD, FAAD Assistant Clinical Professor, Department of Dermatology, Weil Cornell Medical College; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Assistant Attending Dermatologist, New York Presbyterian Hospital; Assistant Attending Dermatologist, Lenox Hill Hospital, North Shore-LIJ Health System; Private Practice

Noah S Scheinfeld, JD, MD, FAAD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Abbvie<br/>Received income in an amount equal to or greater than $250 from: Optigenex<br/>Received salary from Optigenex for employment.

Specialty Editor Board

Michael J Wells, MD, FAAD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Jeffrey Meffert, MD Associate Clinical Professor of Dermatology, University of Texas School of Medicine at San Antonio

Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Timothy McCalmont, MD Director, UCSF Dermatopathology Service, Professor of Clinical Pathology and Dermatology, Departments of Pathology and Dermatology, University of California at San Francisco; Editor-in-Chief, Journal of Cutaneous Pathology

Timothy McCalmont, MD is a member of the following medical societies: Alpha Omega Alpha, American Medical Association, American Society of Dermatopathology, California Medical Association, College of American Pathologists, United States and Canadian Academy of Pathology

Disclosure: Received consulting fee from Apsara for independent contractor.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author, Richard H. Musgnug, MD, to the development and writing of this article.

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Preferred sites of vascular involvement by selected vasculitides.
 
 
 
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