Blue rubber bleb nevus syndrome (Bean syndrome) is a rare, severe disorder of unknown cause, characterized by numerous cutaneous and internal venous malformations; gastrointestinal lesions are pathognomonic. We discovered somatic mutations in TEK, the gene encoding TIE2, in 15 of 17 individuals with blue rubber bleb nevus syndrome. Somatic mutations were also identified in five of six individuals with sporadically occurring multifocal venous malformations. In contrast to common unifocal venous malformation, which is most often caused by the somatic L914F TIE2 mutation, multifocal forms are predominantly caused by double (cis) mutations, that is, two somatic mutations on the same allele of the gene. Mutations are identical in all lesions from a given individual. T1105N-T1106P is recurrent in blue rubber bleb nevus, whereas Y897C-R915C is recurrent in sporadically occurring multifocal venous malformation: both cause ligand-independent activation of TIE2, and increase survival, invasion, and colony formation when expressed in human umbilical vein endothelial cells.

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The ability to predict severity of the post-thrombotic syndrome (PTS) early after acute deep-vein thrombosis (DVT) is limited. The aim of our study was to examine the incidence of PTS prospectively and to evaluate the predictive value of non-invasive venous examinations shortly after DVT for the development of PTS. In 93 patients with DVT thrombosis score (TS), reflux, venous outflow resistance (VOR) and calf muscle pump dysfunction (CMP) were examined prospectively. After one, two and six years patients were evaluated for PTS using the clinical scale of the CEAP-classification (PTS present > or = 3 on a scale from 0 to 6). Area under the curves (AUC) were used to evaluate the predictive value of the non-invasive examinations at one and three months after diagnosis of DVT for future PTS. The cumulative incidence of PTS increased from 49% (32/65) after one year to 55% (36/65) and 56% (27/48) after two and six years, whereas the incidence of patients with PTS class 4 progressed from 20% after two years to 33% after six years. The prognostic value to predict PTS was highest for the combination of TS, VOR and reflux measured three months after diagnosis and showed an AUC of 0.77 (0.65-0.90) for PTS after one year. In conclusion, the incidence of PTS after DVT did not increase significantly after one year, whereas during longer follow-up the severity of PTS rose in patients with PTS. Moreover, measurement of TS, VOR and reflux three months after DVT could predict, with reasonable accuracy, the risk of PTS after one year of follow-up.

Washing without water mildly protects from skin abnormalities/lesions, costs for preparing and performing bed baths do not differ from costs for traditional bed bathing. Thus, washing without water can be considered the more efficient alternative.

Setting: Patients from 12 outpatient dermatology clinics were invited to participate in this study. When they agreed, they were asked to wear an accelerometer for a week and were then interviewed at the outpatient clinic.Patients: A total of 150 patients with leg ulcers caused mainly by venous insufficiency. Main Outcome Measures:The amount of moderately strenuous physical activity, the amount of walking, and adherence to compression therapy.Results: In this study, 39% of the patients interviewed displayed adherence to compression therapy. Selfreported data validated by the use of an accelerometer indicated that the amount of moderately strenuous activity in the study group was low compared with that of the general Dutch population; 35% of the patients did not have a 10-minute walk even once a week.Conclusions: Low levels of physical activity were established in a group of 150 patients with venous leg ulcers. Full adherence to compression therapy was reported in about 40% of the patients. Patients should be educated and encouraged to (1) enhance physical activity through walking and leg exercises and (2) increase adherence to compression therapy.