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A Case Series of 118 Patients

Elisabeth Mazoyer, MD; Odile Enjolras, MD; Annouk Bisdorff, MD; Jérome Perdu, MD; Michel Wassef, MD; Ludovic Drouet, MD

Arch Dermatol. 2008;144(7):861-867.

Objective  To investigate the clinical characteristics of venous malformation of the limbs and trunk and known but poorly appraised associated coagulation disorders. Venous malformations are ubiquitous, slow-flow vascular anomalies known to be occasionally painful because of thrombotic episodes inside the lesion.

Design  Large case series, with screening of accepted standard coagulation tests.

Setting  Ambulatory multidisciplinary clinics for vascular anomalies.

Patients  This 2-year study (2003-2005) included 118 patients with clinical, radiological, and biological features informative for better defining venous malformation and associated coagulation abnormalities.

Main Outcome Measures  The primary outcome was coagulation disorders associated with VM. Secondary measures include anatomic location, extent of lesion, localized pain, and impaired motion.

Results  The mean age of patients was 27 years, and there was a female preponderance of 64%. The venous malformation involved the upper extremity, lower extremity, and trunk in 30%, 58%, and 36% of patients, respectively; it was plurifocal in 22%. Intralesional pain (in 92% of patients) had a higher frequency in female (63%) than in male (47%) patients. Tissular involvement concerned the skin (65%), muscle (73%), bone (13%), joints (12%), and viscera (9%). According to our severity scoring system, cases of less gravity had a score of 2 or 3 (52%), cases of intermediate severity had a score of 4 or 5 (32%), and cases of major severity had a score of 6 to 9 (10%). The most frequent blood coagulation abnormality was a high plasma D-dimer level (> 0.5 µg/mL) (58% of patients), which was correlated with muscle involvement and high severity score and was more frequent in women. The factor VIII–von Willebrand factor complex was documented in 84 patients, and plasma von Willebrand factor level was decreased (<60%) in 23 (27%) of them; 10 of the 84 patients (12%) had more notably decreased levels (<50%).

Conclusions  This study of a large case series of patients with pure venous malformation in the limbs and/or trunk highlights muscle involvement and frequency of pain. It validates that coagulation disorders, present in 58% of our patients, create thrombotic painful events. Under certain circumstances, these disorders entail a risk of hemorrhage because of the progression of localized intravascular coagulopathy to disseminated intravascular coagulopathy.

Author Affiliations: Consultation Multidisciplinaire des Angiomes (Drs Mazoyer, Enjolras, Bisdorff, and Perdu), Service d’Anatomie Pathologique (Dr Wassef), and Service d’Hématologie Biologique (Drs Mazoyer and Drouet), Hôpital Lariboisière, Assistance Publique-Hopitaux de Paris (AP-HP), Université Paris, Paris, France; Service d’Hématologie Biologique, Hôpital Avicenne, AP-HP, Bobigny, France (Dr Mazoyer); Service de Chirurgie Maxillo-Faciale et Chirurgie Plastique, Hôpital d’enfants Armand-Trousseau, AP-HP, Paris (Dr Enjolras); and Laboratoire de Génétique, Hôpital Européen Georges Pompidou, AP-HP, Paris (Dr Perdu).

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