eMedicine Specialties > Dermatology > Diseases of the Vessels

Benign Lymphangioendothelioma

Author: Walter HC Burgdorf, MD, Clinical Lecturer, Department of Dermatology, Ludwig Maximilian University, Munich, Germany
Contributor Information and Disclosures

Updated: Mar 10, 2009

Introduction

Background

Benign lymphangioendothelioma (BLAE) is an uncommon vascular tumor that is of importance primarily because it can be confused histologically with Kaposi sarcoma (KS) or angiosarcoma.1,2 Jones et al first described the tumor as acquired progressive lymphangioma and later as benign lymphangioendothelioma.3 Acquired progressive lymphangioma and benign lymphangioendothelioma are synonymous, and they have no distinguishing clinical features.

Pathophysiology

 Benign lymphangioendothelioma is a proliferation of lymphatic endothelial cells that stain positively with podoplanin (D2-40), LYVE-1, and PORX-1. Benign lymphangioendothelioma is not associated with preexisting vascular malformations or lymphedema. Although the lesion rarely is identified during infancy, some suggest it is a hamartoma that first becomes apparent during adolescence or young adult life; the development of benign lymphangioendothelioma is possibly triggered by hormonal changes.

Frequency

United States

Benign lymphangioendothelioma is rare; fewer than 30 cases have been reported.

Mortality/Morbidity

Benign lymphangioendothelioma is a benign process with no associated mortality and minimal morbidity.

Race

No racial predisposition is reported.

Sex

Males and females are affected equally.

Age

Benign lymphangioendothelioma primarily affects adolescents and young adults.

Clinical

History

A single, asymptomatic, slowly expanding patch, plaque, or nodule usually manifests during adolescence or young adulthood. Congenital lesions can occur.

Physical

Flat to slightly elevated, slowly expanding, red-brown lesions that resemble a bruise may be observed. Occasionally, a red-brown dermal nodule or a skin-colored subcutaneous mass may be observed, either within the patch or alone.4,5



<BR>Erythematous nodule with a hint of erythemato...


Erythematous nodule with a hint of erythematous macular component at periphery.

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<BR>Erythematous nodule with a hint of erythemato...


Erythematous nodule with a hint of erythematous macular component at periphery.


Causes

In most instances, the cause is unknown. Trauma has often been blamed, but a reliable connection has never been established. In one patient, femoral arteriography was a plausible trigger.6 One case has been reported in association with HIV/AIDS.7

More on Benign Lymphangioendothelioma

Overview: Benign Lymphangioendothelioma
Differential Diagnoses & Workup: Benign Lymphangioendothelioma
Treatment & Medication: Benign Lymphangioendothelioma
Follow-up: Benign Lymphangioendothelioma
Multimedia: Benign Lymphangioendothelioma
References
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References

  1. Guillou L, Fletcher CD. Benign lymphangioendothelioma (acquired progressive lymphangioma): a lesion not to be confused with well-differentiated angiosarcoma and patch stage Kaposi's sarcoma: clinicopathologic analysis of a series. Am J Surg Pathol. Aug 2000;24(8):1047-57. [Medline].

  2. Requena L, Sangueza OP. Cutaneous vascular proliferation. Part II. Hyperplasias and benign neoplasms. J Am Acad Dermatol. Dec 1997;37(6):887-919; quiz 920-2. [Medline].

  3. Jones EW, Winkelmann RK, Zachary CB, Reda AM. Benign lymphangioendothelioma. J Am Acad Dermatol. Aug 1990;23(2 Pt 1):229-35. [Medline].

  4. Hwang LY, Guill CK, Page RN, Hsu S. Acquired progressive lymphangioma. J Am Acad Dermatol. Nov 2003;49(5 Suppl):S250-1. [Medline].

  5. Kim HS, Kim JW, Yu DS. Acquired progressive lymphangioma. J Eur Acad Dermatol Venereol. Mar 2007;21(3):416-7. [Medline].

  6. Kato H, Kadoya A. Acquired progressive lymphangioma occurring following femoral arteriography. Clin Exp Dermatol. Mar 1996;21(2):159-62. [Medline].

  7. Paik AS, Lee PH, O'Grady TC. Acquired progressive lymphangioma in an HIV-positive patient. J Cutan Pathol. Nov 2007;34(11):882-5. [Medline].

  8. Herron GS, Rouse RV, Kosek JC, Smoller BR, Egbert BM. Benign lymphangioendothelioma. J Am Acad Dermatol. Aug 1994;31(2 Pt 2):362-8. [Medline].

  9. Watanabe M, Kishiyama K, Ohkawara A. Acquired progressive lymphangioma. J Am Acad Dermatol. May 1983;8(5):663-7. [Medline].

  10. Grunwald MH, Amichai B, Avinoach I. Acquired progressive lymphangioma. J Am Acad Dermatol. Oct 1997;37(4):656-7. [Medline].

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Further Reading

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Keywords

benign lymphangioendothelioma, acquired progressive lymphangioma, BLAE,  Kaposi sarcoma, KS, angiosarcoma, targetoid hemosiderotic hemangioma

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Contributor Information and Disclosures

Author

Walter HC Burgdorf, MD, Clinical Lecturer, Department of Dermatology, Ludwig Maximilian University, Munich, Germany
Walter HC Burgdorf, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, American Society of Dermatopathology, International Society of Dermatopathology, and Society for Pediatric Dermatology
Disclosure: Nothing to disclose.

Medical Editor

Gregory J Raugi, MD, PhD, Professor, Department of Internal Medicine, Division of Dermatology, University of Washington at Seattle; Chief, Dermatology Section, Primary and Specialty Care Service, Veterans Administration Medical Center of Seattle
Gregory J Raugi, MD, PhD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

Pharmacy Editor

David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic
David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Managing Editor

Rosalie Elenitsas, MD, Herman Beerman Associate Professor of Dermatology, University of Pennsylvania School of Medicine; Director, Penn Cutaneous Pathology Services, Department of Dermatology, University of Pennsylvania Health System
Rosalie Elenitsas, MD is a member of the following medical societies: American Academy of Dermatology and American Society of Dermatopathology
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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