Angioendotheliomatosis C84.4
Synonym(s)
History
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Definition
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A very rare disease characterized by multicentric, intravascular tumor cell proliferations. The term “angioendotheliomatosis” has now largely been abandoned in favor of the term “intravascular lymphoma” (see Classification), so that it now has only historical significance.
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Classification
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- Angioendotheliomatosis proliferans maligna: More frequent malignant form, 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.
Tables
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Distinguishing Between the Reactive and Malignant Forms of Angioendotheliomatosis
Criteria |
Angioendotheliomatosis, malignant ( see below: Cutaneous B/T-cell lymphoma, intravascular (large-cell) |
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Pattern of involvement |
Limited to the skin |
Systemic involvement, particularly the central nervous system and internal organs |
Concomitant conditions |
Frequently associated with other conditions, e.g., subacute bacterial endocarditis, food intolerance, malignancy |
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Histology |
In the region of the superior and deep vascular plexuses, multiluminal, glomerular-like vascular and endothelial cell proliferates are present, with lumens that are partly dilated and partly occluded by endothelial cell proliferates. Focal swelling and hyperplasia with enlarged, slightly dyschromatic, and pleomorphic cell nuclei. In the upper vascular plexus, obstruction of the vascular lumina by fibrin thrombi, erythrocytes, or disintegrating granulocytes. |
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Capillary and intravascular endothelial cell proliferations. No intravascular atypical lymphocytes |
Intravascular, pleomorphic B-lymphocytes and, more rarely, T-lymphocytes |
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Immunohistochemistry |
Endothelial cell proliferation (Factor VIII-AG, CD 31 positive) |
Lymphocyte markers in addition to endothelial cell markers |
Prognosis |
Resolution of skin lesions within months following treatment of the underlying disease |
Usually fatal outcome despite treatment |
Literature
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- 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.
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Prof. Dr. med. Peter Altmeyer