Lymphangiectasia Clinical Presentation

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Apr 20, 2012
 

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, or scleroderma. Sometimes, they resolve in weeks without any treatment.[5]

Rarely, lymphangiectasias may occur in pregnancy and spontaneously regress with childbirth.[6]

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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.

Conjunctival lymphangiectasis may be evident as intermittent conjunctival swelling and dilated conjunctival vessels on ocular examination.[7]

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.[8]

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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[9] ; irradiation alone for various malignancies[10] ; metastatic lymph node obstruction; and various scarring processes, such as infections, keloids, scleroderma, and scrofuloderma.[11]

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 cancer.[12, 13, 14, 15]

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.[16]

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Contributor Information and Disclosures
Author

Robert A Schwartz, MD, MPH  Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

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

Disclosure: Nothing to disclose.

Coauthor(s)

Geover Fernandez, MD, FAAD  Staff Physician, Department of Dermatology, University of Medicine and Dentistry New Jersey, New Jersey Medical School

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

Disclosure: Nothing to disclose.

Specialty Editor Board

James Fulton Jr, MD, PhD  Center for Cosmetic Dermatology; Consultant, Vivant Pharmaceuticals, LLC

James Fulton Jr, MD, PhD is a member of the following medical societies: American Academy of Cosmetic Surgery, American Academy of Dermatology, American Society for Laser Medicine and Surgery, Dermatology Foundation, International Society of Cosmetic and Laser Surgeons, and Skin Cancer Foundation

Disclosure: Vivant Pharmaceuticals Grant/research funds Consulting

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, Northside 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.

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: Lippincott Williams Wilkins Royalty Textbook editor; DLA Piper Consulting fee Consulting

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M 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, Ackerman Academy of Dermatopathology, New York

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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  2. Pena JM, Ford MJ. Cutaneous lymphangiectases associated with severe photoaging and topical corticosteroid application. J Cutan Pathol. Apr 1996;23(2):175-81. [Medline].

  3. Back SJ, Kim YJ, Choi DK, et al. Cutaneous lymphangiectasia associated with photoageing and topical corticosteroid application. Clin Exp Dermatol. Apr 2009;34(3):352-4. [Medline].

  4. Shon W, Ida CM, Boland-Froemming JM, Rose PS, Folpe A. Cutaneous angiosarcoma arising in massive localized lymphedema of the morbidly obese: a report of five cases and review of the literature. J Cutan Pathol. Apr 26 2011;[Medline].

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  6. Verma S. Pregnancy-induced lymphangiectasias of the vulva. Int J STD AIDS. Mar 2008;19(3):211-2. [Medline].

  7. Kiliç A, Gül A, Cinal A. Conjunctival Lymphangiectasis. Ophthalmic Surg Lasers Imaging. Apr 2 2010;1-2. [Medline].

  8. Varron L, Vignes S, Green L, Morelec I, Broussolle C, Seve P. Recurrent lymphangiectasia of the left supraclavicular fossa: an unusual cause of paroxystic swelling. Arch Dermatol. Nov 2011;147(11):1337-8. [Medline].

  9. Sener SF, Milos S, Feldman JL, et al. The spectrum of vascular lesions in the mammary skin, including angiosarcoma, after breast conservation treatment for breast cancer. J Am Coll Surg. Jul 2001;193(1):22-8. [Medline].

  10. Schwab RA, McCollough ML. Acquired vulvar lymphangiomas: a sequela of radiation therapy. Cutis. Mar 2001;67(3):239-40. [Medline].

  11. Di Leonardo M, Jacoby RA. Acquired cutaneous lymphangiectasias secondary to scarring from scrofuloderma. J Am Acad Dermatol. Apr 1986;14(4):688-90. [Medline].

  12. Ambrojo P, Cogolludo EF, Aguilar A, Sanchez Yus E, Sanchez de Paz F. Cutaneous lymphangiectases after therapy for carcinoma of the cervix--a case with unusual clinical and histological features. Clin Exp Dermatol. Jan 1990;15(1):57-9. [Medline].

  13. Handfield-Jones SE, Prendiville WJ, Norman S. Vulval lymphangiectasia. Genitourin Med. Oct 1989;65(5):335-7. [Medline].

  14. Landthaler M, Hohenleutner U, Braun-Falco O. Acquired lymphangioma of the vulva: palliative treatment by means of laser vaporization carbon dioxide. Arch Dermatol. Jul 1990;126(7):967-8. [Medline].

  15. Mendiratta V, Harjai B, Sardana K. Tubercular lymphadenitis with lymphangiectases of the vulva. J Eur Acad Dermatol Venereol. Mar 2005;19(2):264-5. [Medline].

  16. Garcia-Doval I, de la Torre C, Losada A, Ocampo C, Rodriguez T, Cruces MJ. Acquired cutaneous lymphangiectasia in a patient with cirrhotic ascites. J Eur Acad Dermatol Venereol. Sep 1999;13(2):109-12. [Medline].

  17. del Pozo J, Martinez W, Yebra-Pimentel MT, Fonseca E. Lymphangiectatic variant of pilomatricoma. J Eur Acad Dermatol Venereol. Sep 2004;18(5):575-6. [Medline].

  18. Shekhel T, Glick RM, Cranmer LD. In-transit metastasis from melanoma presenting as lymphangiectasis: a case report. Cutis. Sep 2009;84(3):151-8. [Medline].

  19. Harwood CA, Mortimer PS. Acquired vulval lymphangiomata mimicking genital warts. Br J Dermatol. Sep 1993;129(3):334-6. [Medline].

  20. Mu XC, Tran TA, Dupree M, Carlson JA. Acquired vulvar lymphangioma mimicking genital warts. A case report and review of the literature. J Cutan Pathol. Mar 1999;26(3):150-4. [Medline].

  21. el Sayed F, Bazex J, Bouissou X, et al. Acquired cutaneous lymphangiectasia mimicking plantar warts. Br J Dermatol. Jun 1995;132(6):1014-6. [Medline].

  22. Ahmed DD, Waldorf JC, Randle HW. Cutaneous lymphangiectasis: treatment with sclerotherapy. Plast Reconstr Surg. Feb 1998;101(2):434-6. [Medline].

  23. Meisler DM, Eiferman RA, Ratliff NB, Burns CD. Surgical management of conjunctival lymphangiectasis by conjunctival resection. Am J Ophthalmol. Oct 2003;136(4):735-6. [Medline].

  24. Fraunfelder FW. Liquid nitrogen cryotherapy for conjunctival lymphangiectasia: a case series. Trans Am Ophthalmol Soc. Dec 2009;107:229-32. [Medline]. [Full Text].

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