Proliferating systematized angioendotheliomatosis

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,…”

“…16,21,27,30,34,39,40,60,88 Lesions were said to have involved both sides of the body in 47 patients. 1,3,5,[7][8][9]13,16,21,25,30,34,37,40,42,44,45,[48][49][50]52,54,56,58,60,61,64,68,69,72,74,82,[87][88][89]93 Most commonly lesions were described as being nodules and ⁄or plaques (49%); macules were encountered in 22AE5% of the patients. Lesions were said to be indurated or firm in 27AE5% of the patients.…”

“…Photographs of clinical lesions were included for only 32 patients, [1][2][3]7,9,10,13,16,19,21,22,24,27,30,34,37,39,45,[48][49][50]52,60,66,68,74,82,87,89 10 of those having IVL-BCL, 34,39,50,60,68,74,82,87,89 six IVL-TCL 37,45,48,52,66 and 16 not being characterized as either TCL or BCL. [1][2][3]7,9,10,13,16,19,21,22,24,27,30,…”

“…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 .…”