Systemic angioendotheliomatosis with metastases.

Abstract

The clinical and pathological findings of a case of systemic angioendotheliomatosis are presented. The previously published cases of this disease are analysed. The present case is only the third to show clear evidence of metastasis.

Skin manifestations of intravascular lymphoma mimic inflammatory diseases of the skin

“…Twenty-nine had lesions restricted to the skin, 1,3,16,22,[25][26][27]40,49,50,54,57,60,68,74,75,87,89,92 whereas in the other 52 patients organs other than the skin were also involved. In 26 of these 52 patients, lesions in the skin preceded those in other organs 5,[7][8][9][10][11]13,21,24,26,30,34,37,39,40,44,48,60,61,64,66,69,93 whereas in the remaining 26, signs and symptoms of other organs preceded the appearance of lesions in the skin. 2,6,17,19,20,24,26,34,…”

“…37,40,57,66 45,81 Data on treatment and follow-up were available for 81 patients. [1][2][3][6][7][8][9][10][11]13,16,20,22,24,25,30,34,36,37,42,44,45,48,[50][51][52]54,[56][57][58]60,61,64,66,69,72,75,81,88,89,92,93 The vast majority of patients received combination chemotherapy such as CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone), and some received radiation treatment either as a single treatment or in combination with chemotherapy. Death from IVL that presented with lesions in the skin was explicitly mentioned in 42 patients and is summarized in Table 3.…”

“…Articles reporting on a total of 224 patients with IVL were reviewed. In articles before 1986 and many thereafter no differentiation is made by the authors between B‐cell and T‐cell lymphoma (105 patients) 1–27,29,30,36,49,51,53,77,92 . Beginning in 1986, authors increasingly included immunohistochemical data of the patients studied by them 19,20,23,25 .…”

“…Intravascular lymphoma (IVL) was described first in 1959 by Pfleger and Tappeiner under the designation 'Angioendotheliomatosis proliferans systemisata'. 1 The disease was for long considered to be a malignant neoplasm of endothelial differentiation [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18] but immunohistochemical studies finally revealed the lymphocytic differentiation of the proliferations of atypical cells within vessels. 19,20 Histopathological findings are stereotypical and consist of infiltrates of large blasts within vessels of the superficial and deep plexus in the dermis sometimes also involving vessels in the upper part of the subcutaneous fat ( Fig.…”

“…Intravascular lymphoma is a rare disease that often has a fatal course because patients do not present with signs and symptoms that allow a correct diagnosis to be made before the disease is well advanced 1–97 . Sometimes, however, skin lesions enable a diagnosis to be made early, but diagnostic criteria for such lesions are not well established, patients being reported almost exclusively in the form of case reports.…”