[Malignant systemic angioendotheliomatosis. Apropos of a case with an ultrastructural and histo-immunologic study]

Clinico-pathological data in a typical case are compared with those of 54 published reports of systemic angioendotheliomatosis. In this rare disease the lesions, characterized by clusters of abnormal cells in the small vessels, usually have multivisceral localizations, with chiefly cutaneous and encephalic manifestations and a rapidly fatal course. However cases are known with lesions restricted to the skin and a protracted if not regressive course; about half of these cases are associated with an endocarditis. In the case reported, the results of ultrastructural and histo-immunological studies (f. VIII, lysozyme, anti-protease, Ig) are in favour of the endothelial origin of the abnormal cells, a now widely accepted hypothesis.