Angioendotheliomatosis C84.4

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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angioendotheliomatosis proliferans; intravascular endothelioma

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Gottron and Nikolowski, 1958

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Very rare disease with multicenter intravascular tumor cell proliferation. Today, the term "angioendotheliomatosis" has largely been abandoned in favour of the term "intravascular lymphoma", so that it only has a historical significance.

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Two forms with similar clinical picture are distinguished:See Table 1.

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Differentiation between reactive and malignant forms of angioendotheliomatosis


Angioendotheliomatosis, reactive (benign)

Angioendotheliomatosis, malignant (see below lymphoma cutaneous B/T-cell lymphoma, intravascular (large cell)

Infestation pattern

Restricted to skin

Systemic infestation, especially of the central nervous system and internal organs

Accompanying illness

Frequently associated with diseases, e.g. subacute bacterial endocarditis, food intolerance, malignancy


In the area of the upper and deep vascular plexus, multiluminal, glomerular vascular and endothelial cell proliferation with partially dilated lumina, partially closed by endothelial cell proliferation. Focal swelling and hyperplasia with enlarged, slightly dyschromic and pleomorphic cell nuclei. In the upper vascular plexus the vascular lumina are blocked with fibrin thrombi, erythrocytes or disintegrating granulocytes.

Capillary and intravascular endothelial cell proliferation. No intravascular atypical lymphocytes

Intravascularly located, pleomorphic B and, more rarely, T lymphocytes


Endothelial cell proliferation (factor VIII-AG, CD 31 positive)

Lymphocyte markers in addition to endothelial cell markers


Healing of the skin changes after treatment of the underlying disease within months

Mostly lethal outcome despite therapy

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  1. Gottron WA, Nikolowski N (1958) Extrarenal Löhlein-Herdnephritis of the skin in endocarditis. Arch Klin Exp Dermatol 207: 156-175
  2. Kimyai-Asadi A et al (1999) Diffuse dermal angiomatosis: a variant of reactive angioendotheliomatosis associated with atherosclerosis. J Am Acad Dermatol 40: 257-259
  3. Kunstfeld R et al (2001) A unique case of a benign disseminated angioproliferation combining features of Kaposi's sarcoma and diffuse dermal angioendotheliomatosis. J Am Acad Dermatol 45: 601-605
  4. Pfleger L et al (1959) On the knowledge of systemized endotheliomatosis of the cutaneous blood vessels (reticuloendotheliomatosis?). Dermatologist 10: 359
  5. Satzger I (2009) Intravascular B-cell lymphomas. Dermatologist 60: 131-136
  6. Schley G et al (2001) Angioendotheliomatosis proliferans systematisata. Dermatologist 52: 1030-1034
  7. Schmidt K et al (1996) Reactive angioendotheliomatosis in chronic lymphatic leukemia Dermatologist 47: 550-555
  8. Thai KE et al (2003) Reactive angioendotheliomatosis in the setting of antiphospholipid syndrome. Australas J Dermatol 44: 151-155


Please ask your physician for a reliable diagnosis. This website is only meant as a reference.


Last updated on: 29.10.2020