Cutaneous collagenous vasculopathy (CCV) is a rare cutaneous microangiopathy that clinically resembles
generalized essential telangiectasia with only 12 cases reported to date. The perivascular
fibrosis is thought to be due to production of abnormal
collagen by veil cells in the outer vessel walls as a result of unknown factors. This report is of an 84-year-old male with progressive
telangiectasia. Biopsies showed characteristic features of CCV. In addition, there were multiple intravascular
fibrin thrombi, some organizing and associated with endothelial cell
hyperplasia with recanalization reminiscent of glomeruloid bodies and simulating
reactive angioendotheliomatosis (RAE). Histochemically and ultrastructurally
fibrin was noted within the vessel walls integrating into the fibrous tissue around the vessels; however, the patient had no evidence of coagulation disorder,
cryoglobulinemia or cold agglutinemia. Immunofluorescence showed
fibrinogen within the vessel walls but no
immunoglobulins or C3. As well, there were minimal inflammatory cells. This suggests pauci-inflammatory injury to the endothelial cells by unknown angiogenic factors causing local intravascular
fibrin thrombi with
fibrin leaking and incorporating into the vessel walls, eventually leading to reparative perivascular
fibrosis. This case suggests that some cases of CCV are related to a primary local intravascular occlusive
thrombotic microangiopathy. However, the primary triggering factor causing the endothelial cell damage has yet to be elucidated.