Synonym(s)
HistoryThis section has been translated automatically.
DefinitionThis section has been translated automatically.
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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ClassificationThis section has been translated automatically.
- Angioendotheliomatosis proliferans maligna: More frequent, malignant form of the disease, which histologically can be regarded as intravascular T-cell or B-cell lymphoma. More frequent is the intravascular B-cell lymphoma, less frequently the intravascular T-cell lymphoma.
- Angioendotheliomatosis, reactive: Very rare, benign form with exclusive skin involvement, healing spontaneously or after therapy of the underlying disease.
TablesThis section has been translated automatically.
Differentiation between reactive and malignant forms of angioendotheliomatosis
Criteria |
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 |
|
Histology |
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 |
|
Immunohistology |
Endothelial cell proliferation (factor VIII-AG, CD 31 positive) |
Lymphocyte markers in addition to endothelial cell markers |
Forecast |
Healing of the skin changes after treatment of the underlying disease within months |
Mostly lethal outcome despite therapy |
LiteratureThis section has been translated automatically.
- Gottron WA, Nikolowski N (1958) Extrarenal Löhlein-Herdnephritis of the skin in endocarditis. Arch Klin Exp Dermatol 207: 156-175
- Kimyai-Asadi A et al (1999) Diffuse dermal angiomatosis: a variant of reactive angioendotheliomatosis associated with atherosclerosis. J Am Acad Dermatol 40: 257-259
- 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
- Pfleger L et al (1959) On the knowledge of systemized endotheliomatosis of the cutaneous blood vessels (reticuloendotheliomatosis?). Dermatologist 10: 359
- Satzger I (2009) Intravascular B-cell lymphomas. Dermatologist 60: 131-136
- Schley G et al (2001) Angioendotheliomatosis proliferans systematisata. Dermatologist 52: 1030-1034
- Schmidt K et al (1996) Reactive angioendotheliomatosis in chronic lymphatic leukemia Dermatologist 47: 550-555
- Thai KE et al (2003) Reactive angioendotheliomatosis in the setting of antiphospholipid syndrome. Australas J Dermatol 44: 151-155
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Outgoing links (3)
Diffuse dermal angiomatosis ; Intravascular cutaneous t-cell lymphoma; Intravascular large b-cell lymphoma;Disclaimer
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.
Prof. Dr. med. Peter Altmeyer