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Lymphangiectasia Clinical Presentation

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Jun 22, 2016
 

History

Patients with lymphangiectasia typically present with numerous fluid-filled vesicles in a chronic lymphedematous area several years after surgery, more commonly due to a malignancy. The cutaneous lesions of lymphangiectasia can range from clear, fluid-filled blisters to smooth, flesh-colored nodules, often appearing along an incisional scar.

Coexisting lymphedema is present in most patients with acquired lymphangioma.

Patients can present with localized wetness or copious drainage of clear or milky fluid from ruptured vesicles. Pain and recurrent cellulitis are complications associated with lymphangiectasia.

Acquired lymphangiectases may be associated upper limb lymphedema secondary to mastectomy, radiotherapy, keloids, chronic lymphedema, or scleroderma.[5, 6] Sometimes, they resolve in weeks without any treatment.[7]

Rarely, lymphangiectasias may occur in pregnancy and spontaneously regress with childbirth.[8] Acquired lymphangiectasia of the glans may also occur after circumcision.[9]

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Physical

Clinically, lymphangiectasia consists of several clusters of translucent, thick-walled, fluid-filled vesicles. The vesicles typically measure 2-10 mm in diameter. The affected area appears to be speckled by numerous translucent vesicles with normal-appearing skin among the lesions. Some lymphangiectasia lesions may become pedunculated with a hyperkeratotic verrucous surface mimicking a wart.[10]

Conjunctival lymphangiectasis may be evident as intermittent conjunctival swelling and dilated conjunctival vessels on ocular examination.[11]

Although many patients without chronic lymphedema have been reported, it is a common physical finding in patients with acquired lymphangioma. Diffuse infiltration of subcutaneous tissue by lymphangiectasia may produce painless swelling at sites such as the subclavicular fossa.[12]

Lymphangiectasia may also be evident on the penis. Benign transient lymphangiectasis of the penis may be evident.[13] Acquired lymphangiectasia of the glans may occur after circumcision.[9]

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Causes

Acquired lymphangiomas can arise from a large number of external factors that cause structural damage to previously normal deep lymphatics.

Lymphangiectases have been reported following radical mastectomy with or without radiation therapy[14] ; irradiation alone for various malignancies[15] ; metastatic lymph node obstruction; and various scarring processes, such as infections, keloids, scleroderma, and scrofuloderma.[16]

Lymphangiectases have been described in the penis and the scrotum after removal of a sacrococcygeal tumor; they may also arise on the vulva and the inner thigh after surgery for cervical or other pelvic cancers.[17, 18, 19, 20] Pelvic lymphatic obstruction may produce acquired vulvar lymphangiomas.[21]

Acquired lymphangiomas have been reported in the genital region of elderly patients without evidence of lymphatic obstruction.

Garcia-Doval et al reported lymphangiectasis in a patient with cirrhotic ascites.[22]

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Contributor Information and Disclosures
Author

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.

Coauthor(s)

Geover Fernandez, MD, FAAD Staff Physician, Department of Dermatology, Rutgers New Jersey Medical School

Geover Fernandez, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society for MOHS Surgery

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: American Medical Association, Alpha Omega Alpha, Association of Military Dermatologists, American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Phi Beta Kappa

Disclosure: Nothing to disclose.

Rosalie Elenitsas, MD Herman Beerman 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, American Medical Association, American Society of Dermatopathology, Pennsylvania Academy of Dermatology

Disclosure: Received royalty from Lippincott Williams Wilkins for textbook editor.

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.

References
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