Treatment for Livedoid Vasculopathy: A Systematic Review

JAMA Dermatol. 2018 Feb 1;154(2):193-202. doi: 10.1001/jamadermatol.2017.4374.

Abstract

Importance: Livedoid vasculopathy is a painful, ulcerative condition of the lower extremities for which no established treatment exists. Current treatment paradigms rely on low levels of evidence, primarily case reports and case series.

Objective: To systematically review the treatment for livedoid vasculopathy and synthesize the available clinical data.

Evidence review: A systematic review of the literature using Ovid MEDLINE (covering the period January 1, 1946, through June 9, 2017) and Ovid EMBASE (covering January 1, 1947, through June 9, 2017) databases was performed with a broad and inclusive search strategy along with a subsequent search of the references of retrieved articles. All case series reports published in the English language and in a peer-reviewed journal discussing the treatment for livedoid vasculopathy diagnosis were included.

Findings: A total of 29 case series reports published in the English language and in a peer-reviewed journal discussed the treatment for livedoid vasculopathy. These reports represented a total of 339 patients, of whom 230 (68%) were female and 69 (20%) were male; sex was not stated for 40 patients. Treatment with anticoagulants, antiplatelets, anabolic steroids, thrombolytics, hyperbaric oxygen, intravenous immunoglobulins, vitamin supplementation, UV light, and a combination of 1 or more of these among other therapies had a favorable outcome. Anticoagulants were the most commonly used monotherapy, achieving a favorable response in 62 of 63 patients (98%). Anabolic steroids, intravenous immunoglobulins, and antiplatelets were the second, third, and fourth most commonly used treatments, respectively. All of these therapies were associated with good clinical outcomes. Adverse events were observed in 44 patients (13%).

Conclusions and relevance: A variety of treatments with varying degrees of success have been used to treat livedoid vasculopathy. Randomized clinical trials should be performed in the future to better establish these treatments in clinical practice.

Publication types

  • Systematic Review

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Anticoagulants / pharmacology
  • Anticoagulants / therapeutic use*
  • Combined Modality Therapy
  • Female
  • Humans
  • Hyperbaric Oxygenation / methods
  • Immunoglobulins, Intravenous / administration & dosage
  • Livedo Reticularis / diagnosis
  • Livedo Reticularis / therapy*
  • Male
  • PUVA Therapy / methods
  • Prognosis
  • Risk Assessment
  • Severity of Illness Index
  • Skin Diseases, Vascular / diagnosis
  • Skin Diseases, Vascular / therapy*
  • Treatment Outcome

Substances

  • Adrenal Cortex Hormones
  • Anticoagulants
  • Immunoglobulins, Intravenous