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  • Title: [Systemic proliferative angioendotheliomatosis: a cutaneous manifestation of malignant B-cell lymphomas. Histologic and immunohistologic studies of two cases].
    Author: Kutzner H, Englert W, Hellenbroich D, Embacher G, Kutzner U, Schröder J.
    Journal: Hautarzt; 1991 Jun; 42(6):384-90. PubMed ID: 1917462.
    Abstract:
    Angioendotheliomatosis proliferans systemisata (AEPS) is a rare disease entity characterized by a predominantly intravascular proliferation of tumour cells. Two forms of AEPS are differentiated: a very rare, benign and self-limiting form, which is endothelial in origin, and a more common, malignant form, which is an angiotropic intravascular malignant B-cell lymphoma. Histological and immunohistological investigations of the malignant form of AEPS are presented: In a 69-year-old woman cutaneous lesions appeared 5 months before the diagnosis of B-immunoblastic lymphoma. In a 57-year-old woman lesions were observed simultaneously with the relapse of a high-grade malignant B-cell lymphoma. Immunohistological identification of the proliferating cell type made diagnosis of intravascular B-cell lymphoma possible in paraffin-embedded biopsies.
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