[Angioendotheliomatosis associated with chronic venous insufficiency].

Reactive angioendotheliomatosis (RAE) is the name introduced by Tappeiner and Pfleger in 1963 to refer to a rare condition characterized by a benign proliferation of endothelial cells. It has been observed in patients with concurrent systemic diseases, in whom vascular occlusion or underlying vascular disease favors a reactive proliferation of endothelial cells. Presentation varies from multiple foci of erythematous macules, ecchymoses, or purpuric plaques to ulcerated plaques affecting the trunk, limbs, or face.1 Histology shows vascular proliferation with obliteration of the lumina secondary to endothelial cell hyperplasia and noninflammatory microthrombosis. The differential diagnosis must include benign and malignant vascular tumors, particularly Kaposi sarcoma and angiosarcoma. RAE has no specific treatment; it tends to be self-limiting and resolves spontaneously or after treatment of the underlying disorder. We present the case of a 68-year-old woman who consulted for the progressive appearance of violaceous plaques on the skin of the left leg. The plaques were completely asymptomatic and had gradually enlarged over several months. Examination revealed multiple erythematous-purpuric macules and plaques on the left leg. The lesions had an irregular outline with an atrophic center that was more yellow in color and on palpation they had an infiltrated, fibrous texture. The clinical findings were suggestive of Kaposi sarcoma (Figure 1). The patient’s history included chronic venous insufficiency treated surgically 30 years earlier by varicose vein stripping in the left leg and sclerotherapy in the same leg 20 years later. Based on this history, we also considered a diagnosis of stasis dermatitis. Hematoxylin-eosin staining of a histology specimen showed a vascular proliferation of capillaries lined by prominent endothelial cells, with microthrombi that occluded the vascular lumen; there was no pleomorphism or nuclear atypia and there were few mitoses. The findings were suggestive of reactive angioendotheliomatosis (Figure 2A). Immunohistochemistry was positive for CD31, confirming the vascular origin of the proliferative cells (Figure 2B), and was negative for CD68. Additional studies, including complete blood count, biochemistry, coagulation, antiphospholipid antibodies, hepatitis serology, rheumatoid factor, protein electrophoresis, and immunoelectrophoresis, were normal or negative. The patient was diagnosed with RAE associated with chronic venous insufficiency; the injection of varicose vein sclerosant could have played an additional pathogenic role. The clinical course during follow-up was favorable, with stability, spontaneous regression, and persistence of discrete brownish macules at revision after 1 year. RAE is a rare disorder characterized by the presence of