Proliferating Angioendotheliomatosis has historically been divided into two groups, (1) a reactive, involuting type and (2) a malignant, rapidly fatal type.[1]: 598
| Proliferating angioendotheliomatosis | |
|---|---|
| Specialty | Oncology  |
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
editProliferating angioendotheliomatosis may be divided into two types:[2]
- a reactive type – Reactive angioendotheliomatosis
- a malignant type – Intravascular large B-cell lymphoma
Treatment
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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
editReferences
edit- ^ a b James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. ISBN 0-7216-2921-0.
- ^ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 978-1-4160-2999-1.