Angioendotheliomatosis Clinical Presentation

Updated: May 14, 2019
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
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History

Patients with the malignant and reactive form may have constitutional symptoms at the time of presentation. Patients most commonly complain of low-grade fever and myalgia. Other complaints include the following:

  • Chills

  • Night sweats

  • Weakness

  • Weight loss

  • Malaise

  • Arthralgia

  • Depression

Skin papules and nodules that are slowly increasing in size can be painful and tender, sometimes with a burning sensation.

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Physical Examination

Similar skin lesions can be observed in both the malignant and the reactive forms. [25] Erythematous-to-violaceous macules, papule, nodules, or plaques are often observed in the abdominal region or the lower extremities. The trunk, arms, breasts, and face, including earlobes, can also be affected. The lesions may be indurated, hemorrhagic, or ulcerative. In the reactive form, lesions are always confined to the skin. In the malignant form, the nervous system seems to be the favorite target of the disease. Apart from that, the following organs are most frequently involved: adrenal glands, thyroid, pancreas, lungs, liver, spleen, lymph nodes, heart, stomach, and kidneys. Bone marrow is typically not affected.

In the malignant form, skin lesions are noted in about 30% of the patients. They tend to localize on the lower extremities and the abdomen.

Aguayo-Leiva et al, Rozenblat et al, and Corti et al reported cellulitislike plaques. [26, 27, 28] CNS signs are observed in about 85% of patients. Adrenal gland involvement may lead to hypoadrenalism. Lymph nodes are generally spared; thus, adenopathy is absent.

Patients rarely present first with primary lung or prostate disease, disseminated intravascular coagulation, lytic bone lesions, or panhypopituitarism.

In diffuse dermal angiomatosis, pulses over the arteries located distally from the site of occlusion can be impalpable.

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Complications

Infarction is a complication if the coronary arteries are involved.

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