Proliferating angioendotheliomatosis

Proliferating angioendotheliomatosis
SpecialtyOncology 
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Proliferating Angioendotheliomatosis has historically been divided into two groups, (1) a reactive, involuting type and (2) a malignant, rapidly fatal type.[1]:598

The reactive involuting type, reactive Angioendotheliomatosis is an rare cutaneous condition characterized histologically by a dense proliferation of small capillaries, and occurs in people with various diseases including subacute bacterial endocarditis and end-stage atherosclerotic disease. These people present with various skin lesions and rashes - most commonly on the thighs. Treatment aimed at the underlying condition hastens the resolution of the lesions.

The malignant type is an intravascular lymphoma, usually of the diffuse B-cell type, known as intravascular large B-cell lymphoma. It progresses rapidly through involvement of multiple body systems and mortality occurs in less than a year from the initial diagnosis. The average age of diagnosis being 55 years. The causative mechanism is unknown. In a few cases treatment with palliative chemotherapy has been effective.[1]:598

Classification of Proliferating Angioendotheliomatosis

Proliferating angioendotheliomatosis may be divided into two types:[2]

Treatment

In few cases palliative chemotherapy is effective.

A 30 year old woman was diagnosed with cutaneous proliferating angioendotheliomatosis. She was treated with a local excision and radiotherapy.

See also

References

  1. 1 2 James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. ISBN 0-7216-2921-0.
  2. Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.


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