Ehlers-Danlos syndrome

Abstract

Ehlers-Danlos syndrome (EDS) is a group of genetically determined vascular purpura. The pathological changes mainly involve joints, skin and the vascular wall. The incidence of the EDS is 1 per 20 000-100 000 births. The defects involve the structure of collagen as well as other enzymes associated with it. Today there are the following types of EDS: classical, hypermobility, vascular, kyphoscoliotic, arthrochalasia and dermatosparaxis. The individual types of EDS are accompanied by complications of heart muscle, respiratory system, gastrointestinal tract, eyes, teeth, periodontal tissues and obstetric problems. The current paper presents issues relating to epidemiology, pathogenesis, clinical manifestations and treatment of various forms of Ehlers-Danlos syndrome. © Copyright by Wroclaw Medical University.