Angioendotheliomatosis reactive (benign) D21.9

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

angioendotheliomatosis benigna; Angioendotheliomatosis inflammatory; Diffuse dermal angiomatosis; inflammatory angioendotheliomatosis; reactive angioendotheliomatosis

History
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Nurse and Tappeiner 1959

Definition
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Reactive, self-limited, real inflammatory, lymphoreticular disease that can occur in the context of systemic diseases such as bacterial endocarditis, rheumatoid arthritis, cryoglobulinemia, and monoclonal gammopathy. The disease is an expression of an intravalous endothelial proliferation, which is triggered by fibrin thrombi or cryoprotein thrombi.

The reactive angioendotheliomatosis must be distinguished from the malignant form of angioendotheliomatosis, which is understood as intravascular lymphoma(primary cutaneous intravascular large cell lymphoma).

Occurrence/Epidemiology
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Very rare; < 100 cases are described in world literature. No sex preference.

Etiopathogenesis
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Cryoglobulins, cold agglutinins and phospholipid antibodies were detected in a group of patients. In other cases, endoprophetic surgery was performed. A release of angiogenesis factors is assumed within the framework of the underlying disease.

Localization
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Mostly located on the trunk and extremities.

Clinical features
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Disease pattern limited to the skin, multifaceted and thus clinically not very groundbreaking. Mostly formation of solitary, asymptomatic, blurred, reticular or homogeneous extensive redness. But also disseminated, indurated, small or large plaques have been described, as well as subcutaneous nodules. More rare are papular or plaque-shaped exanthema interspersed with petechiae, sometimes with the aspect of a livedo.

Histology
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Proliferation of densely packed solid and luminaceous capillaries that form networks in places. The vascular cavities are lined with clumsy endothelial cells that protrude into the lumens. No cell atypia, no increased proliferation tendency of the endothelial proliferates.

Diagnosis
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Clinic, histology, immunohistology.

Differential diagnosis
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  • Malignant angioendotheliomatosis (is regarded as intravascular T-cell or B-cell lymphoma; histological differentiation; systemic involvement)
  • Polyarteritis nodosa, cutaneous: palpable purpura, rare live images, urticarial exanthema, subcutaneous nodules with pyoderma gangraenosum-like ulcerations. Often pulmo-renal syndrome.
  • Erythema nodosum: acute event; painfulness. Localization mainly on the lower legs.
  • Early forms of Mycosis fungoides: Histology is diagnostic.
  • Kaposi's sarcoma (reddish-brown colour, located in the tension lines of the skin)
  • Angiosarcoma: Mostly occurring in older people; development here in the area of long-standing lymphedema (e.g. following mastectomy and lymphadenectomy), after radiotherapy and immune defects (e.g. HIV infection). If angiosarcomas occur after radiotherapy, the radiations are usually at least 5-6 years ago; longer intervals are not uncommon and can last for decades.
  • Lymphangiosis carcinomatosa of visceral neoplasia: rough clinically mostly uncharacteristic plaques.

Therapy
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Symptomatic

External therapy
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Wound cleansing and hydrocolloid dressings. Consistent protection against cold.

Internal therapy
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Symptomatic

Progression/forecast
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After treatment of the underlying disease: spontaneous healing under external wound therapy after a few months is possible. Recurrences are possible after 1-2 years at the latest.

Literature
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  1. Kleimann P (2017) Reactive angioendotheliomatosis. Nude Dermatol 44: 463-464
  2. Lazova RC et al (1996) Reactive angioendotheliomatosis. Amer J Dermatopathol 18: 63-69
  3. Mayor Ibarguren A et al (2014) Diffuse Reactive Angioendotheliomatosis
  4. Secondary to the Administration of Trabectedin and Pegfilgrastim. At J Dermatopathol PubMed PMID: 25140665.
  5. Satzger I et al (2009) Intravascular B-cell lymphomas. Dermatologist 60: 131-136
  6. Schmidt K et al (1996) Reactive angioendotheliomatosis in chronic lymphocytic leukemia. Dermatologist 47: 550-555
  7. Tollefson MM et al (2014) Diffuse dermal angiomatosis of the breast: Clinicopathologic study of 5 patients. J Am Acad Dermatol PubMed PMID: 25264238
  8. Wagner G (2007) Reactive angioendotheliomatosis after implantation of a shoulder joint prosthesis. Act Dermatol 33: 42-46
  9. Wallenfang K, Stadler R (2002) Angioendotheliomatosis. Nude Dermatol 28: 374-379
  10. Willemze R et al (2005) WHO-EORTC classification for cutaneous lymphomas. Blood 105: 3768-3785

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Last updated on: 29.10.2020