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Reactive Angioendotheliomatosis

Last updated May 16, 2018

Approved by: Maulik P. Purohit MD, MPH

Reactive Angioendotheliomatosis (RAE) is rare, but benign condition that is marked by the presence of red rashes on skin, mostly on the chest and back. It is associated with a host of diseases and disorders.

What are the other Names for this Condition? (Also known as/Synonyms)

  • RAE (Reactive Angioendotheliomatosis)

What is Reactive Angioendotheliomatosis? (Definition/Background Information)

  • Reactive Angioendotheliomatosis (RAE) is rare, but benign condition that is marked by the presence of red rashes on skin, mostly on the chest and back. It is associated with a host of diseases and disorders
  • The condition may be described as a reaction of the skin to the underlying disorder, through abnormal proliferation of blood vessels/capillaries. The underlying conditions may include heart diseases, sarcoidosis, rheumatoid arthritis, and many others
  • Reactive Angioendotheliomatosis is mostly observed in adults. It may be linked to an immunocompromised health status of the individual. Healthcare providers often describe RAE as an indicator of an underlying health condition
  • The treatment and prognosis of the condition is related to the underlying cause of Reactive Angioendotheliomatosis. In many cases, RAE resolves spontaneously on its own within a few months

Who gets Reactive Angioendotheliomatosis? (Age and Sex Distribution)

  • Reactive Angioendotheliomatosis is mostly observed during adulthood (mostly between 40-85 years). It is extremely uncommon in children
  • This skin condition can affect both males and females uniformly
  • There is no known ethnic or racial preference

What are the Risk Factors for Reactive Angioendotheliomatosis? (Predisposing Factors)

The following conditions are known to be associated with Reactive Angioendotheliomatosis:

  • Heart diseases involving the valves
  • Sarcoidosis
  • Antiphospholipid syndrome
  • Polymyalgia rheumatic
  • Liver cirrhosis or liver disease
  • Cryoglobulinemia
  • Rheumatoid arthritis
  • Glioblastoma multiforme
  • Arteriovenous fistula caused due to a medical treatment/surgery (iatrogenic)
  • Peripheral vascular atherosclerosis
  • Immunocompromised status, such as due to an organ transplant

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Reactive Angioendotheliomatosis? (Etiology)

  • The exact cause of Reactive Angioendotheliomatosis is unknown
  • They are thought to occur as a reactive process to an existing underlying health condition. Many individuals have a poor immune system at the time of onset of this skin condition
  • Even though the underlying or associated condition may affect the entire body (systemic), RAE only affects the skin

What are the Signs and Symptoms of Reactive Angioendotheliomatosis?

The signs and symptoms of Reactive Angioendotheliomatosis may vary from one individual to another. The presentations may also depend upon the underlying condition and the health status of the individual.

  • Any part of the body may be affected, although the usual sites are the arms and legs and the trunk
  • The skin lesions may be in the form of papules, plaques, macules, or nodules of different sizes
  • Almost all individuals present reddish or inflamed skin rashes; the reddish discoloration occurs from the presence of numerous capillaries on the skin surface that results in increased blood supply to the area
  • The lesions may be widespread on the body or localized/confined to a region; localized lesions are generally associated with iatrogenic arteriovenous fistula or peripheral vascular atherosclerosis
  • Bleeding and skin ulceration is very rarely observed
  • The period of presentation of the rashes may be between a month to several years
  • Signs and symptoms of the underlying systemic condition may be noted

How is Reactive Angioendotheliomatosis Diagnosed?

A diagnosis of Reactive Angioendotheliomatosis would involve:

  • Physical examination with evaluation of the individual’s medical history
  • Dermoscopy: It is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
  • Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using ultraviolet light. It is performed to examine the change in skin pigmentation
  • Skin biopsy: A biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis
  • Appropriate tests and procedures to establish any underlying condition/disorder
  • A differential diagnosis to eliminate the following may be undertaken prior to establishing a definitive diagnosis:
    • Kaposi sarcoma
    • Tufted angioma
    • Intravascular histiocytosis

Note: A skin biopsy may not be necessary in most cases, but may be performed to rule out other conditions with similar signs and symptoms.

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Reactive Angioendotheliomatosis?

  • Typically, there are no complications associated with Reactive Angioendotheliomatosis per se
  • However, complications that arise from the underlying condition may be noted

How is Reactive Angioendotheliomatosis Treated?

  • Generally, no treatment is needed for Reactive Angioendotheliomatosis, since it occurs as a reaction to an underlying condition
  • In many cases, Reactive Angioendotheliomatosis is self-limiting and is known to disappear spontaneously
  • Undertaking treatment of the underlying condition/disorder (once a diagnosis is established) is necessary; suitable treatment of the condition may result in regression of RAE
  • Follow-up care with regular screening and check-ups are important

How can Reactive Angioendotheliomatosis be Prevented?

  • Current medical research have not established a method of preventing Reactive Angioendotheliomatosis
  • Regular medical screening at periodic intervals with blood tests, scans, and physical examinations, may be advised for those who have this condition

What is the Prognosis of Reactive Angioendotheliomatosis? (Outcomes/Resolutions)

  • The prognosis of Reactive Angioendotheliomatosis is linked to the prognosis of the underlying condition it is associated with. Conditions with mild signs and symptoms have better overall prognosis than those with severe signs and symptoms
  • Most RAE are known to regress and recede on its own, over a period of few weeks to months

Additional and Relevant Useful Information for Reactive Angioendotheliomatosis:

Please visit our Skin Care Health Center for more physician-approved health information:


What are some Useful Resources for Additional Information?

References and Information Sources used for the Article:

Helpful Peer-Reviewed Medical Articles:

Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: Feb. 7, 2017
Last updated: May 16, 2018