Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Angioendotheliomatosis Clinical Presentation

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

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.

Next

Physical

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.

Previous
Next

Causes

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.

Previous
 
 
Contributor Information and Disclosures
Author

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.

Coauthor(s)

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.

References
  1. Echeverría-García B, Botella-Estrada R, Requena C, Guillén C. [Intralymphatic histiocytosis and cancer of the colon]. Actas Dermosifiliogr. 2010 Apr. 101(3):257-62. [Medline].

  2. Bakr F, Webber N, Fassihi H, Swale V, Lewis F, Rytina E, et al. Primary and secondary intralymphatic histiocytosis. J Am Acad Dermatol. 2014 May. 70(5):927-33. [Medline].

  3. Kimyai-Asadi A, Nousari HC, Ketabchi N, Henneberry JM, Costarangos C. Diffuse dermal angiomatosis: a variant of reactive angioendotheliomatosis associated with atherosclerosis. J Am Acad Dermatol. 1999 Feb. 40(2 Pt 1):257-9. [Medline].

  4. Krell JM, Sanchez RL, Solomon AR. Diffuse dermal angiomatosis: a variant of reactive cutaneous angioendotheliomatosis. J Cutan Pathol. 1994 Aug. 21(4):363-70. [Medline].

  5. Yang H, Ahmed I, Mathew V, Schroeter AL. Diffuse dermal angiomatosis of the breast. Arch Dermatol. 2006 Mar. 142(3):343-7. [Medline].

  6. Adams BJ, Goldberg S, Massey HD, Takabe K. A cause of unbearably painful breast, diffuse dermal angiomatosis. Gland Surg. 2012 Aug 1. 1(2):[Medline].

  7. McMenamin ME, Fletcher CD. Reactive angioendotheliomatosis: a study of 15 cases demonstrating a wide clinicopathologic spectrum. Am J Surg Pathol. 2002 Jun. 26(6):685-97. [Medline].

  8. Lomater C, Gerloni V, Gattinara M, Mazzotti J, Cimaz R, Fantini F. Systemic onset juvenile idiopathic arthritis: a retrospective study of 80 consecutive patients followed for 10 years. J Rheumatol. 2000 Feb. 27(2):491-6. [Medline].

  9. Tomasini C, Soro E, Pippione M. Angioendotheliomatosis in a woman with rheumatoid arthritis. Am J Dermatopathol. 2000 Aug. 22(4):334-8. [Medline].

  10. Creamer D, Black MM, Calonje E. Reactive angioendotheliomatosis in association with the antiphospholipid syndrome. J Am Acad Dermatol. 2000 May. 42(5 Pt 2):903-6. [Medline].

  11. Kawaoka J, McBean J, Li H, Mihm MC Jr, Kroumpouzos G. Coexistence of diffuse reactive angioendotheliomatosis and neutrophilic dermatosis heralding primary antiphospholipid syndrome. Acta Derm Venereol. 2008. 88(4):402-3. [Medline].

  12. Agusti A, Hernández P, Oliver V, García R, Alegre de Miquel V. [Angioendotheliomatosis associated with chronic venous insufficiency]. Actas Dermosifiliogr. 2010 Jun. 101(5):464-6. [Medline].

  13. Eming SA, Sacher C, Eich D, Kuhn A, Krieg T. Increased expression of VEGF in glomeruloid reactive angioendotheliomatosis. Dermatology. 2003. 207(4):398-401. [Medline].

  14. Gottron HA, Nikolowski W. [Extrarenal Lohlein focal nephritis of the skin in endocarditis]. Arch Klin Exp Dermatol. 1958. 207(2):156-76. [Medline].

  15. Brazzelli V, Baldini F, Vassallo C, et al. Reactive angioendotheliomatosis in an infant. Am J Dermatopathol. 1999 Feb. 21(1):42-5. [Medline].

  16. Chang A, Zic JA, Boyd AS. Intravascular large cell lymphoma: a patient with asymptomatic purpuric patches and a chronic clinical course. J Am Acad Dermatol. 1998 Aug. 39(2 Pt 2):318-21. [Medline].

  17. Aguayo-Leiva I, Vano-Galvan S, Salguero I, et al. Reactive angioendotheliomatosis in a patient with Myelodysplastic Syndrome presenting as a cellulitis-like plaque. Eur J Dermatol. 2009 Mar-Apr. 19(2):182-3. [Medline].

  18. Rozenblat M, Pessach Y, Gat A, Bergman R, Sprecher E, Goldberg I. Reactive angioendotheliomatosis presenting as cellulitis. Clin Exp Dermatol. 2013 Oct. 38(7):748-50. [Medline].

  19. Corti MA, Rongioletti F, Borradori L, Beltraminelli H. Cutaneous reactive angiomatosis with combined histological pattern mimicking a cellulitis. Dermatology. 2013. 227(3):226-30. [Medline].

  20. Requena L, Fariña MC, Renedo G, Alvarez A, Yus ES, Sangueza OP. Intravascular and diffuse dermal reactive angioendotheliomatosis secondary to iatrogenic arteriovenous fistulas. J Cutan Pathol. 1999 Mar. 26(3):159-64. [Medline].

  21. González-Pérez R, de Lagrán ZM, Soloeta R, Saracíbar N. Reactive angioendotheliomatosis following implantation of a knee metallic device. Int J Dermatol. 2014 Apr. 53(4):e304-5. [Medline].

  22. Mayor-Ibarguren A, Gómez-Fernández C, Beato-Merino MJ, González-Ramos J, Rodríguez-Bandera AI, Herranz-Pinto P. Diffuse Reactive Angioendotheliomatosis Secondary to the Administration of Trabectedin and Pegfilgrastim. Am J Dermatopathol. 2014 Aug 19. [Medline].

  23. Boyapati A, Khan S, Mar A, Sheridan A. Reactive angioendotheliomatosis associated with cyroglobulinemia in a marathon runner. Dermatol Online J. 2013 Nov 15. 19(11):20404. [Medline].

  24. Kaffenberger BH, Zuo RC, Gru A, Plotner AN, Sweeney SA, Devine SM, et al. Graft-versus-host disease-associated angiomatosis: A clinicopathologically distinct entity. J Am Acad Dermatol. 2014 Oct. 71(4):745-53. [Medline]. [Full Text].

  25. Palmer MJ, Lee A, O'Keefe R. Cutaneous reactive angiomatosis associated with erythema ab igne. Australas J Dermatol. 2013 Nov 25. [Medline].

  26. Barnett CR, Seo S, Husain S, Grossman ME. Intravascular B-cell lymphoma: the role of skin biopsy. Am J Dermatopathol. 2008 Jun. 30(3):295-9. [Medline].

  27. Kirke S, Angus B, Kesteven PJ, Calonje E, Simpson N. Localized reactive angioendotheliomatosis. Clin Exp Dermatol. 2007 Jan. 32(1):45-7. [Medline].

  28. Fukunaga M. Expression of D2-40 in lymphatic endothelium of normal tissues and in vascular tumours. Histopathology. 2005 Apr. 46(4):396-402. [Medline].

  29. Mensing CH, Krengel S, Tronnier M, Wolff HH. Reactive angioendotheliomatosis: is it 'intravascular histiocytosis'?. J Eur Acad Dermatol Venereol. 2005 Mar. 19(2):216-9. [Medline].

 
Previous
Next
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.