Cutaneous collagenous vasculopathy (CCV) is a rare form of acquired cutaneous telangiectasia. This disorder was originally described as idiopathic microangiopathy of small cutaneous blood vessels, associated with generalized telangiectasia [1,2]. This disorder is almost always clinically diagnosed as primary essential telangiectasia and the correct diagnosis is only made following skin biopsies although the microscopic features can be very subtle and missed.

There are now at least 25 cases described in the English literature [3], but only one and possibly another case show intravascular microthrombi associated with organization [4,5]. This together with the marked thickening and reduplication of the basement membrane characteristic of CCV suggests repeated local endothelial cell damage as the primary event leading to reparative fibrosis by connective tissue cells in the outer vessel walls.

Although the cause of the endothelial cell injury is not known, it is possible that genetic defects (or other factors) are responsible, similar to other fibrosing disorders. Future research should be hopefully directed at finding the triggering factor leading to the microvascular endothelial injury.