Increased Expression of VEGF in Glomeruloid Reactive Angioendotheliomatosis

Abstract

Reactive angioendotheliomatosis (RAE) is a very rare disorder characterized by marked proliferation of endothelial cells. It is often associated with infections, such as subacute bacterial endocarditis, but has also been described as an early sign of a developing hematological malignancy. We report the case of a 71-year-old Caucasian female who developed lupus-like RAE lesions. A thorough diagnostic workup and subsequent 3-year clinical follow-up revealed no sign of an underlying infectious or neoplastic disorder. Repetitive serum immunofixations were only once consistent with a monoclonal gammopathy of undetermined significance. In lesional skin, the pronounced bud-like endothelial cell formation was associated with an increased epidermal expression of vascular endothelial growth factor (VEGF), a potent angiogenic mediator. In accordance with the paracrine action of epidermally derived VEGF, vascular endothelial cells in lesional skin revealed increased expression of the VEGF receptor VEGFR-2 (KDR). This case suggests a possible role of epidermally derived VEGF in endothelial cell proliferation in RAE.

Incidental Simultaneous Finding of Intravascular Histiocytosis and Reactive Angioendotheliomatosis

“…A summary of the clinical and histopathologic features of these entities is presented in Table 1. 3,4,[9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][28][29][30][31][32][33][34][35][36][37][38] Our case supports the previous proposal that both RAE and IH are actually 2 different aspects of the same Majority: B cells phenotype; CD20 + , CD79a + , MUM1 + (95%), bcl-2 + (91%), CD3 2 ; Rare: T/NK-cell phenotype 35 Immunofluorescence findings Granular deposits of IgA, IgM, and complement may be found around the vessels and at the DEJ 34 …”

“…Review of the literature reveals less than 50 cases. [1][2][3][4][9][10][11][12][13][14][15][16][17][18][19][20][21][22] Most of them were associated with infections such as acute otitis media and pulmonary tuberculosis, coexistent systemic diseases (renal disease, valvular cardiac disease, alcoholic cirrhosis, and rheumatoid arthritis/polymyalgia rheumatica), blood disorders (myelodysplastic syndrome, cryoproteinemias, monoclonal gammopathies, antiphospholipid syndrome), and vascular abnormalities (iatrogenic arteriovenous fistulas, severe peripheral vascular atherosclerotic disease). 9,[11][12][13][14][15][16][17][18][19][20][21][22] The number of reports of IH in the literature is similar to those of RAE.…”

Cutaneous Reactive Angiomatosis Associated With Chronic Lymphoid Leukemia

“…12 In the 2 reported CRA cases associated to CLL, cutaneous lesions appeared 3 years previously to CLL development in one case 10 and 6 years after the diagnosis in the other one. 2 In fact, it has been proposed that cutaneous angiomatosis may be a potential cutaneous marker of B-CLL.…”

“…7 Several systemic diseases have been reported associated with CRA, 8 including plasma cell and lymphoproliferative disorders. 2,6,7,[9][10][11][12] We report a case of CRA with unusual histopathological findings, associated with chronic lymphoid leukemia (CLL).…”

Glomeruloid Hemangiomas in the Absence of POEMS Syndrome

“…In 2003, Eming et al demonstrated increased VEGF expression in the epidermis and increased expression of VEGF receptor 2 in glomeruloid hemangioma. The proximity of the two suggests that VEGF exhibits paracrine activity 7 . In 2007, increased VEGF expression and VEGF receptor 1 expression was documented by immunohistochemistry in a glomeruloid hemangioma from a patient with POEMS syndrome, which additionally suggests autocrine function by VEGF 8 .…”