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Angioendotheliomatosis Clinical Presentation

  • Author: Anna Zalewska, MD, PhD; Chief Editor: Dirk M Elston, MD  more...
Updated: Oct 10, 2014


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.



Similar skin lesions can be observed in both the malignant and the reactive forms.[16] 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.[17, 18, 19] 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.



Different triggers (eg, subacute bacterial endocarditis; circulating immune complexes; fibrin; cholesterol emboli; viruses, such as hepatitis C; atriovenous fistula[20] ; atherosclerotic emboli; trauma; metal implants[21] ; drugs such as trabectedin and pegfilgrastim administered for recurring myxoid liposarcoma[22] ) should be kept in mind in reactive angioendotheliomatosis.

Other reported associations include cryoglobulinemia,[23] graft versus host disease,[24] and erythema ab igne.[25]

In diffuse dermal angiomatosis (a form of reactive angioendotheliomatosis), ischemia induces a local increase of VEGF, a well-known inducer of endothelial cell proliferation. In hypoxia, such situations can occur in different tissues.

Contributor Information and Disclosures

Anna Zalewska, MD, PhD Professor of Dermatology and Venereology, Psychodermatology Department, Chair of Clinical Immunology and Microbiology, Medical University of Lodz, Poland

Disclosure: Nothing to disclose.


Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Paul Krusinski, MD Director of Dermatology, Fletcher Allen Health Care; Professor, Department of Internal Medicine, University of Vermont College of Medicine

Paul Krusinski, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Arash Taheri, MD Research Fellow, Center for Dermatology Research, Department of Dermatology, Wake Forest University School of Medicine

Disclosure: Nothing to disclose.

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