eMedicine Specialties > Dermatology > Diseases of the Vessels

Lymphangiectasia

Author: Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Coauthor(s): Geover Fernandez, MD, FAAD, Staff Physician, Department of Dermatology, University of Medicine and Dentistry New Jersey, New Jersey Medical School
Contributor Information and Disclosures

Updated: Nov 13, 2009

Introduction

Background

Lymphangiectases represent superficial lymphatic dilatation caused by a wide range of scarring processes. Lymphangiectasia occurs as a consequence of lymphatic damage by an external cause, leading to obstruction of local lymphatic drainage. Lymphangiectases are also termed acquired lymphangiomas. Acquired lymphangiomas most commonly occur in adults as a late sequela of mastectomy and radiation therapy. Patients usually present with numerous translucent vesicles in a chronic lymphedematous area several years after surgery with or without radiation therapy.

Nomenclature has not been consistent.1 Some authors apply the terms acquired lymphangioma and lymphangioma circumscriptum interchangeably. In both conditions, the typical cutaneous lesions are groups of small translucent vesicles, often compared with frog spawn. Although both share similar clinical and histologic features, the authors believe that they are 2 distinct entities. The term acquired lymphangioma (lymphangiectasia) is used when dilated lymphatic channels arise following damage to previously normal deep lymphatics, whereas lymphangioma circumscriptum is used when lymphatic channel dilation occurs because of congenital malformations of the lymphatic system involving the skin and the subcutaneous tissues.

Pathophysiology

The pathogenesis of lymphangiectasia is not known; however, the vesicles associated with lymphangiectasia are suggested to represent saccular dilations of local superficial lymphatics. These vesicles develop secondary to increased intralymphatic pressure as a result of buildup of lymph in the superficial vessels caused by damage to previously normal deep lymphatics. This mechanism explains the accompanying lymphedema seen in most patients with lymphangiectasia. The lymphedema usually arises as a result of obstructed lymphatic drainage after mastectomy, radiation therapy, or tumor mass compression. Lymphangiectasia may also result from abnormal dermal structure and function, possibly related to photoaging and steroid-related atrophy.2,3

Frequency

United States

The true incidence of lymphangiectasia is not known. Although lymphangiectasia has been reported in the literature with increased frequency in the past 2 decades, the disease remains rare.

Mortality/Morbidity

  • Lymphangiectasia is a nonfatal disease associated with a high tendency for local recurrence after treatment. Lymphangiectasia may be complicated by chronic copious drainage, pain, and recurrent bouts of cellulitis. In addition, lesions are often cosmetically undesirable.
  • Acquired lymphangiomas are not believed to have malignant potential, although associated chronic lymphedema places the patient at risk for lymphangiosarcoma (Stewart-Treves syndrome), which is an aggressive tumor with a dismal prognosis.

Race

No racial predominance has been reported for lymphangiectasia.

Sex

No sexual predominance has been reported for lymphangiectasia.

Age

In contrast to lymphangioma circumscriptum, acquired lymphangioma is more common in adults than in children. More generally, the condition occurs in patients between their fifth and seventh decades of life.

Clinical

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.4
  • Rarely, lymphangiectasias may occur in pregnancy and spontaneously regress with childbirth.5

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.
  • Although many patients without chronic lymphedema have been reported, it is a common physical finding in patients with acquired lymphangioma.

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 therapy6 ; irradiation alone for various malignancies7 ; metastatic lymph node obstruction; and various scarring processes, such as infections, keloids, scleroderma, and scrofuloderma.8
  • 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.9,10,11,12
  • 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.13

More on Lymphangiectasia

Overview: Lymphangiectasia
Differential Diagnoses & Workup: Lymphangiectasia
Treatment & Medication: Lymphangiectasia
Follow-up: Lymphangiectasia
References
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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. Valdes F, Peteiro C, Toribio J. [Acquired lymphangiectases and breast cancer]. Actas Dermosifiliogr. Jun 2007;98(5):347-50. [Medline].

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

  6. 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].

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

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

  9. 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].

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

  11. 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].

  12. 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].

  13. 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].

  14. 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].

  15. 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].

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

  17. 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].

  18. 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].

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

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  23. Hunt SJ, Santa Cruz DJ. Vascular tumors of the skin: a selective review. Semin Diagn Pathol. Aug 2004;21(3):166-218. [Medline].

  24. Loche F, Schwarze HP, Bazex J. Treatment of acquired cutaneous lymphangiectasis of the thigh and vulva with a carbon dioxide laser. Acta Derm Venereol. Jul 1999;79(4):335. [Medline].

  25. Mallett RB, Curley GK, Mortimer PS. Acquired lymphangioma: report of four cases and a discussion of the pathogenesis. Br J Dermatol. Apr 1992;126(4):380-2. [Medline].

  26. Moon SE, Youn JI, Lee YS. Acquired cutaneous lymphangiectasia. Br J Dermatol. Aug 1993;129(2):193-5. [Medline].

  27. Noel AA, Gloviczki P, Bender CE, Whitley D, Stanson AW, Deschamps C. Treatment of symptomatic primary chylous disorders. J Vasc Surg. Nov 2001;34(5):785-91. [Medline].

  28. Peixoto CA, Figueiredo-Silva J. Fine structure of intrascrotal lymphatic vessels infected by Wuchereria bancrofti adult worms. J Submicrosc Cytol Pathol. Jan-Apr 2001;33(1-2):125-31. [Medline].

  29. Ziv R, Schewach-Millet M, Trau H. Lymphangiectasia. A complication of thoracotomy for bronchial carcinoid. Int J Dermatol. Mar 1988;27(2):123. [Medline].

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Further Reading

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Keywords

lymphangiectasis, lymphangiectases, acquired lymphangioma, acquired lymphangiectasia, secondary lymphangioma, lymphangioma circumscriptum

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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, UMDNJ-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.

Medical Editor

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 Ownership interest Consulting

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, 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.

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 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, 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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