Systemic endotheliomatosis: a case report.

Abstract

A 48-year-old Caucasian man was found to have a rare neoplastic process of vascular endothelial origin. The clinical presentation was that of plum-colored cutaneous plaques of the lower extremities. The histopathology, natural history, and treatment of this case are discussed and compared with previously reported cases. It is suggested that this disease entity represents a malignant state with systemic involvement and a poor prognosis.

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,…”

“…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.…”

“…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.…”

Systemic angioendotheliomatosis with metastases.

“…(M), skull bones (M), brain (V), spleen (M) Shtern and Likhachev (1963) Brain Haber et al (1964) Skin,? abdomen (clinical signs only) Midana and Ormea (1965) Skin Strouth et al (1965) Brain (V), adrenal (V) Abulafia et al (1969) Skin (V arteries and veins) Fievez et al (1971) Skin; capillaries in most of body Okagaki and Richart (1971) Genital tract (V) Madara et al (1975) Skin; capillaries of adbominal organs Scott et al (1975) Skin puzzling feature of the present case is the failure to find the tumour cells in the peripheral blood. A series of buffy coat films collected between the time of amputation and death were examined without success.…”