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Anne Dompmartin, MD; Fanny Ballieux; Pascal Thibon, MD; Agnès Lequerrec, MPhSc; Cédric Hermans, MD, PHD; Philippe Clapuyt, MD; Marie-Thérèse Barrellier, MD; Franck Hammer, MD; Daniel Labbé, MD; Miikka Vikkula, MD, PhD; Laurence M. Boon, MD, PhD

Arch Dermatol. 2009;145(11):1239-1244.

Objective  To evaluate if elevated D-dimer level is specific for venous malformations (VMs) and thus useful for differential diagnosis, which can be problematic even in specialized interdisciplinary centers. Localized intravascular coagulopathy, characterized by elevated D-dimer levels, has been observed in approximately 40% of patients with VMs.

Design  Prospective convenience sample accrued from 2 interdisciplinary sites.

Setting  Two interdisciplinary centers for vascular anomalies in Brussels, Belgium, and Caen, France

Participants  The study population comprised 280 patients with clinical data, Doppler ultrasonograms (for 251 patients), and coagulation parameter measurements.

Main Outcome Measure  Measurement of D-dimer levels.

Results  A VM was diagnosed in 195 of 280 patients (69.6%), and 83 of them had elevated D-dimer levels; the sensitivity of D-dimer dosage was 42.6% (95% confidence interval, 35.6%-49.5%). Among the 85 patients without VM, D-dimer levels were elevated only in 3 patients; the specificity of the dosage was 96.5% (95% confidence interval, 92.5%-100%).

Conclusions  Elevated D-dimer level is highly specific for VMs (pure, combined, or syndromic), and therefore this easy and inexpensive biomarker test should become part of the clinical evaluation of vascular anomalies. It can detect hidden VMs and help differentiate glomuvenous malformation (normal D-dimer levels) from other multifocal venous lesions. Elevated D-dimer level also differentiates a VM from a lymphatic malformation. Moreover, slow-flow Klippel-Trenaunay syndrome (capillaro-lymphatico-venous malformation with limb hypertrophy) can be distinguished from fast-flow Parkes Weber syndrome (capillary malformation with underlying multiple microfistulas and limb hypertrophy). For these reasons, D-dimer level measurement is a useful complementary tool for diagnosing vascular anomalies in everyday practice.

Author Affiliations: Departments of Dermatology (Drs Dompmartin and Barrellier), Hygiene (Dr Thibon), Hematology (Dr Lequerrec), and Plastic Surgery (Dr Labbé), Université de Caen Basse Normandie, Centre Hospitalier Universitaire de Caen, Caen, France; and Center for Vascular Anomalies, Division of Plastic Surgery (Ms Ballieux and Dr Boon), Hematosis and Thrombosis Unit, Division of Haematology & Laboratory of Thrombosis and Haemostasis, Department of Biological Chemistry (Dr Hermans), Department of Radiology (Drs Clapuyt and Hammer), and Laboratory of Human Molecular Genetics, de Duve Institute (Drs Vikkula and Boon), Université catholique de Louvain, Brussels, Belgium.

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