Parkes Weber syndrome

Parkes Weber syndrome (F. Parkes Weber; synonym: syndrome of Klippel - Trenaunay, osteoreparation varicose nevus, hypertrophic generictake) is characterized by the following three symptoms. 1. Flat angioma skin, single or multiple, different size and shape, located more often unilaterally on the face, trunk or limbs. 2. Widespread or limited expansion and hypertrophy of the venous (less arterial) blood vessels and capillaries with education angiomatous growths on the bottom (at least upper limbs. Between surface and deep vessels are arterio-venous anastomoses. 3. Hyperplasia of the soft tissues and bones of the affected limb (a symptom of a non-permanent). Limb lengthening a few inches and increases in volume, the skin on it condenses, with some difficulty folds of fat, skin temperature and sweating increased, develops hypertrichosis; places are marked valikoobraznye, protruding above the skin swelling bluish-red colour, formed due to the expansion of veins. While long walk limb swells. As a complication may develop ulcers.
A congenital disease, often manifests itself in childhood. The pathogenesis is unclear. Some authors to highlight congenital developmental delay and dysplasia vessels, bones and soft tissue, others change tissues due hypervascularization, third - primary lesion sympathetic nodes and vegetative centers, causing tissue, including vascular changes.
The prognosis is poor due to the gradual development of heart failure. There are various trophic disorders tissue of the limbs, injuries can occur bleeding; there is also the susceptibility vascular wall of thrombogenesis.
Treatment. Excision or dressing dilated blood vessels and Anastasiya branches. Electrocoagulation and effective radiotherapy in cases where the vascular process is limited. To remove the capillary angiomas shown cryotherapy.