You have been logged out of VisualDx or your session has expired.

Please reload this page and sign into VisualDx to continue.

  VisualDx Mobile   Select Language

Get VisualDx Mobile

There are VisualDx mobile apps available for iOS and Android devices.

You will need a VisualDx account to use the mobile apps.

Already have an account? Sign In or
sign up for a free trial.

Users with VisualDx accounts earn CME credits for using VisualDx.

Already have an account? Sign In or
sign up for a free trial.

Create a Personal Account

E-mail (username)
Verify Password
First Name
Last Name

Personal Account Created

Mobile Access

You can now download VisualDx for your iOS and Android devices. Launch the VisualDx app from your device and sign in using your VisualDx personal account username and password.

CME Certification

Sign in with your personal account to earn and claim CME credits through VisualDx. Credits can be earned by building a differential or looking up a diagnosis.

Version: 7.13.1441   (build df7aed4)
Select Language

Select Region

Send us your feedback

This field is required

Oops! There was an issue during submission. Please try again. If the problem persists, email with your feedback.

Thank You!

We appreciate your feedback and you will be hearing from us soon.


Share This Page

Thank You!

We have sent an e-mail with a link to the current page.


E-mail This Patient Information Sheet

Thank You!

We have sent an e-mail with this patient information.


Image Contributors


  • Christine Ahn MD
    Carl Allen DDS, MSD
    Brandon Ayres MD
    Howard P. Baden MD
    Robert Baran MD
    Keira Barr MD
    Gregory J. Basura MD, Ph.D
    Donald Belsito MD
    Jeffrey D. Bernhard MD
    Jesse Berry MD
    Victor Blanco MD
    Benjamin R. Bohaty MD
    William Bonnez MD
    Sarah Brenner MD
    Robert A. Briggaman MD
    Robert Brodell MD
    Roman Bronfenbrener MD
    Walter Brooks MD
    William Buckley MD
    Philip Bulterys MD, PhD (candidate)
    Susan Burgin MD
    Sonya Burton MD
    Sean P. Bush MD, FACEP
    Jeffrey Callen MD
    Scott Camazine MD
    Michael Cardwell
    Shelley D. Cathcart MD
    Robert Chalmers MD, MRCP, FRCP
    Chia-Yu Chu MD, PhD
    Flavio Ciferri MD
    Maria Rosa Cordisco MD
    Noah Craft MD, PhD
    John T. Crissey MD
    Harold E. Cross MD, PhD
    Charles E. Crutchfield III MD
    Adriana Cruz MD
    Donna Culton MD, PhD
    Bart J. Currie MBBS, FRACP, DTM&H
    Chicky Dadlani MD
    Alexander Dane DO
    C. Ralph Daniel III MD
    Thomas Darling MD, PhD
    William Delaney MD
    Damian P. DiCostanzo MD
    Ncoza Dlova MD
    James Earls MD
    Libby Edwards MD
    Melissa K. Egge MD
    Charles N. Ellis MD
    Rachel Ellis MD
    David Elpern MD
    Nancy Esterly MD
    Stephen Estes MD
    E. Dale Everett MD
    Janet Fairley MD
    David Feingold MD
    Jennifer J. Findeis-Hosey MD
    Benjamin Fisher MD
    Henry Foong MBBS, FRCP
    David Foster MD, MPH
    Brian D. Foy PhD
    Michael Franzblau MD
    Vincent Fulginiti MD
    Sunir J. Garg MD, FACS
    Kevin J. Geary MD
    Lowell Goldsmith MD, MPH
    Sethuraman Gomathy MD
    Bernardo Gontijo MD, PhD
    Kenneth Greer MD
    Kenneth G. Gross MD
    Alan Gruber MD
    Nathan D. Gundacker MD
    Akshya Gupta MD
    Vidal Haddad MSC, PhD, MD
    Edward Halperin MD, MA
    Ronald Hansen MD
    John Harvey
    Rizwan Hassan MD
    Michael Hawke MD
    Jason E. Hawkes MD
    Peter W. Heald MD
    David G. Hicks MD
    Sarah Hocker DO
    Ryan J. Hoefen MD, PhD
    Li-Yang Hsu MD
    William Huang MD
    Sanjana Iyengar MD
    Alvin H. Jacobs MD
    Saagar Jadeja MD
    Shahbaz A. Janjua MD
    Joshua J. Jarvis MD
    Kit Johnson
    Zachary John Jones MD
    Robert Kalb MD
    A. Paul Kelly MD
    Henry Kempe MD
    Loren Ketai MD
    Sidney Klaus MD
    Ashwin Kosambia MD
    Jessica A. Kozel MD
    Carl Krucke
    Mario E. Lacouture MD
    Joseph Lam MD
    Alfred T. Lane MD
    Edith Lederman MD
    Nahyoung Grace Lee MD
    Pedro Legua MD, PhD
    Robert Levin MD
    Bethany Lewis MD
    Sue Lewis-Jones FRCP, FRCPCH
    Taisheng Li MD
    Christine Liang MD
    Shari Lipner MD, PhD
    Adam Lipworth MD
    Jason Maguire MD
    Mark Malek MD, MPH
    Jere Mammino DO
    Ricardo Mandojana MD
    Lynne Margesson MD
    Thomas J. Marrie MD
    Maydel Martinez MD
    Ralph Massey MD
    Patrick McCleskey MD
    Karen McKoy MD
    Thomas McMeekin MD
    Josette McMichael MD
    Somchai Meesiri MD
    Joseph F. Merola MD
    Mary Gail Mercurio MD
    Anis Miladi MD
    Larry E. Millikan MD
    Dan Milner Jr. MD
    Zaw Min MD
    Stephanie Montero
    Alastair Moore MD
    Keith Morley MD
    Dean Morrell MD
    Samuel Moschella MD
    Rehan Naseemuddin MD
    Taimor Nawaz MD
    Vic Newcomer MD
    John Nguyen MD
    Matilda Nicholas MD
    Thomas P. Nigra MD
    Steven Oberlender MD, PhD
    Maria Teresa Ochoa MD
    Art Papier MD
    Lawrence Parish MD
    Tanner Parrent MD
    Mukesh Patel MD
    Lauren Patty-Daskivich MD
    David Peng MD, MPH
    Robert Penne MD
    Nitipong Permpalung MD
    Miriam Pomeranz MD
    Doug Powell MD
    Harold S. Rabinovitz MD
    Christopher J. Rapuano MD
    Sireesha Reddy MD
    Angela Restrepo MD, PhD
    Bertrand Richert MD, PhD
    J. Martin Rodriguez, MD, FACP
    Theodore Rosen MD
    Misha Rosenbach MD
    Scott Schiffman MD
    Robert H. Schosser MD
    Glynis A. Scott MD
    Carlos Seas MD, MSc
    Deniz Seçkin MD
    Daniel Sexton MD
    Paul K. Shitabata MD
    Tor Shwayder MD, FAAP, FAAD
    Elaine Siegfried MD
    Gene Sienkiewicz MD
    Christye Sisson
    Philip I. Song MD
    Mary J. Spencer MD, FAAP
    Lawrence B. Stack MD
    Sarah Stein MD
    William Van Stoecker MD
    Frances J. Storrs MD
    Erik J. Stratman MD
    Lindsay C. Strowd MD
    Erika Summers MD
    Belinda Tan MD, PhD
    Robert Tomsick MD
    Hensin Tsao MD, PhD
    Richard P. Usatine MD
    Jenny Valverde MD
    Vishalakshi Viswanath MD
    Susan Voci MD
    Lisa Wallin ANP, FCCWS
    Douglas Walsh MD
    Ryan R. Walsh MD
    George Watt MD
    Clayton E. Wheeler MD
    Sally-Ann Whelan MS, NP, CWOCN
    Jan Willems MD, PhD
    James Henry Willig MD, MPH
    Karen Wiss MD
    Vivian Wong MD, PhD
    Sook-Bin Woo MS, DMD, MMSc
    Jamie Woodcock MD
    Stephen J. Xenias MD
    Nathaniel Yohannes
    Lisa Zaba MD
    Vijay Zawar MD
    Bonnnie Zhang MD
    Carolyn Ziemer MD
    Jeffrey P. Zwerner MD, PhD


  • Am. Journal of Trop. Med & Hygiene
  • Armed Forces Pest Management Board
  • Blackwell Publishing
  • Bugwood Network
  • Centers For Disease Control and Prevention
  • Centro Internacional de Entrenamiento e Investigaciones Mèdicas (CIDEIM)
  • Dermatology Online Journal
  • East Carolina University (ECU), Division of Dermatology
  • International Atomic Energy Agency
  • Massachusetts Medical Society
  • Oxford University Press
  • Radiological Society of North America
  • Washington Hospital Center
  • Wikipedia
  • World Health Organization
ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferencesView all Images (10)
Other Resources UpToDate PubMed


Print Images (10)
Contributors: Lana N. Kashlan MD, Jag Bhawan MD, Jeffrey D. Bernhard MD
Other Resources UpToDate PubMed


Angioendotheliomatosis is a cutaneous vascular proliferation of endothelial cells within blood vessels. It includes benign and malignant forms.

Reactive angioendotheliomatosis:
Benign angioendotheliomatosis, also known as reactive angioendotheliomatosis, is a rare self-limited condition that can occur at any age. Most cases are idiopathic; however, reactive angioendotheliomatosis has been known to occur in association with subacute bacterial endocarditis, cryoglobulinemia, hepatic disease, monoclonal gammopathy, chronic lymphocytic leukemia, renal disease, rheumatoid arthritis, antiphospholipid antibody syndrome, and atherosclerotic disease. It is hypothesized to occur in response to a circulating capillary growth factor or perhaps a growth factor released in response to tissue hypoxia. In addition to the skin findings of erythematous papules and plaques, there may be constitutional symptoms such as fever, chills, malaise, and weight loss with this disorder.

Angiotropic lymphoma:
What was historically referred to as malignant angioendotheliomatosis is now more properly known as intravascular large cell lymphoma or angiotropic lymphoma. The misnomer angioendotheliomatosis stems from the fact that this condition is characterized by an intraluminal proliferation of large atypical cells that were thought to be derived from endothelial cells. Advances in immunohistochemistry demonstrated that these neoplastic cells are of lymphoid origin, and while cases of T-cell origin have been reported, the most recent literature supports that the majority of cases are B-cell proliferations. There is a slight tendency toward increased incidence among males, and the average age of presentation is 70 years. Precise incidence is uncertain, as most of the literature on this condition has been in the form of case reports and small case series. This condition is aggressive and rapidly fatal.

Fever and central nervous system (CNS) symptoms are the most common presenting symptoms. Because of the vague presenting symptoms and the rapid course, about half of reported cases are diagnosed only on autopsy. Patients often have an elevated serum lactate dehydrogenase (LDH) and erythrocyte sedimentation rate (ESR), anemia, thrombocytopenia, and leukopenia.

About 26% of patients present with only cutaneous symptoms. This variant is almost exclusively seen in women with an average age of presentation of 59 years. Most patients present with disseminated and advanced disease. Additionally, an Asian variant has been described that occurs predominantly but not exclusively in Japan and is characterized by multi-organ failure, hepatosplenomegaly, pancytopenia, and hemophagocytic syndrome.

Cases arising in the setting of other malignancies have been reported. Although the precise incidence is unknown, one series of 38 patients found 16% had either a history of or a concomitant distinct malignancy. This includes both hematopoietic and solid tumors. Approximately one-third of these cases represent non-Hodgkin lymphomas, with diffuse large B-cell lymphomas being the most common. Cases of small lymphocytic lymphomas, follicular lymphomas, and mucosa-associated lymphoid tissue lymphomas have been reported.

There are no known risk factors for angioendotheliomatosis.


C85.80 – Other specified types of non-Hodgkin lymphoma, unspecified site

255102004 – Angioendotheliomatosis

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

Differential of reactive angioendotheliomatosis:
  • Glomeruloid hemangioma – Multiple red-purple dome-shaped papules scattered over the trunk and proximal extremities; occurs in association with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes).
  • Lobular capillary hemangioma (pyogenic granuloma) – Friable, rapidly growing red papule that frequently ulcerates.
  • Dabska tumor – Rare, slow-growing angiosarcoma in children; presents as painless intradermal red-blue nodule.
  • Papillary endothelial hyperplasia – Solitary firm masses that occur most commonly within veins of head and neck.
Differential of angiotropic lymphoma:

Best Tests

Subscription Required

Management Pearls

Subscription Required


Subscription Required


Subscription Required

Last Updated: 10/27/2017
Copyright © 2018 VisualDx®. All rights reserved.
Print 10 Images
View all Images (10)
(with subscription)
Angioendotheliomatosis : Reticular - netlike, Blanching patch
Clinical image of Angioendotheliomatosis
Copyright © 2018 VisualDx®. All rights reserved.