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eMedicine Specialties > Dermatology > Diseases of the Vessels

Lymphangiectasia

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

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

Differential Diagnoses

Dermatitis Herpetiformis
Metastatic Carcinoma of the Skin
Herpes Simplex
Stewart-Treves Syndrome
Herpes Zoster
Warts, Genital
Lymphangioma
Warts, Nongenital
Malignant Melanoma

Other Problems to Be Considered

A lymphangiectatic variant of pilomatricoma has been described.14
Malignant melanoma in-transit metastasis may occasionally resemble lymphangiectasis.15
Lymphangiectasia can mimic different types of warts (eg, plantar, genital).16,17,18

Workup

Other Tests

  • Immunohistochemistry
    • This study is important in differentiating lymphangiomas from hemangiomas in difficult cases.
    • Factor VIII–related antigen testing demonstrates positive results in the endothelial cells of hemangiomas but negative or weakly positive results in the endothelium of lymphangiomas.
    • Ulex europaeus I testing results are positive in the endothelial cells of lymphangiectases and hemangiomas.
    • Immunoperoxide staining with QBEnd10 (anti-CD34) antibodies shows positive reactivity results only in endothelial cells lining the blood vessels of the dermis.

Procedures

  • Skin biopsy: The diagnosis of lymphangiectasia is primarily based on clinical history and conventional light microscopy findings.

Histologic Findings

On microscopic examination, lymphangiectases are characterized by large, dilated lymph vessels lined by a single layer of endothelial cells. They are usually found in the papillary dermis and the reticular dermis. Involvement in the deeper dermis is rare. The overlying epidermis is usually associated with acanthosis and hyperkeratosis but is less marked than in angiokeratomas. At times, acquired lymphangiectases grow above the level of adjacent skin.

Lymphangiectases lack the subcutaneous muscle-coated cisternae characteristic of lymphangioma circumscriptum. Although no specific histologic criteria exist with which to differentiate lymphangioma circumscriptum from lymphangiectasia, some believe that the absence of subcutaneous cisternae in lymphangiectases is a true histologic difference and that this finding reflects the difference in the pathogenesis of both disorders.

Treatment

Medical Care

  • No medical care has been proven to be effective for acquired lymphangiomas because the responsible lymphatic vessels must be either excised or sealed to prevent recurrence.
  • Treatment for lymphangiectases can be difficult, but it is important because of the risk that ruptured vesicles may provide a portal of entry for infection and subsequent cellulitis. To prevent superinfection of ruptured vesicles, cleansing the affected area daily with topical antibacterial agents and applying mupirocin ointment or silver sulfadiazine cream are advisable.

Surgical Care

  • Many surgical treatment modalities have been advocated in the care of lymphangiectases; these modalities include electrodesiccation, laser therapy,11 sclerotherapy,19 cryotherapy, and surgical excision.20
  • Daily compression through bandaging or hosiery, in accessible areas, has yielded acceptable results.

Follow-up

Further Inpatient Care

  • At times, severe recurrent cellulitis may warrant hospitalization in patients with lymphangiectasia, especially in patients who are immunocompromised. Intravenous antibiotic is required in patients with severe cellulitis.

Further Outpatient Care

  • Follow-up care is essential for early treatment of lymphangiectasia recurrences.
  • Lymphangiosarcoma (Stewart-Treves syndrome) may occur in chronic edematous limbs, and early detection is critical.

Complications

  • Lymphangiectases are often complicated by pain, copious fluid drainage, and recurrent attacks of cellulitis.

Prognosis

  • Lymphangiectasia has a good prognosis because most conditions respond well to treatment modalities.

Patient Education

  • Patients should be reassured.
  • Lymphangiectases pose no potential for malignant transformation.
  • For excellent patient education resources, visit eMedicine's Women's Health Center and Cancer and Tumors Center. Also, see eMedicine's patient education articles Mastectomy and Breast Cancer.

Miscellaneous

Medicolegal Pitfalls

  • Poor follow-up care can result in the failure to detect recurrences and to provide early treatment for them.
  • Failure to provide the appropriate treatment for ruptured vesicles can result in severe cellulitis, especially in patients who are immunocompromised.

References

  1. Verma SB. Lymphangiectasias of the skin: victims of confusing nomenclature. Clin Exp Dermatol. Jul 2009;34(5):566-9. [Medline].

  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].

  20. 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].

  21. Celis AV, Gaughf CN, Sangueza OP, Gourdin FW. Acquired lymphangiectasis. South Med J. Jan 1999;92(1):69-72. [Medline].

  22. Harwood CA, Mortimer PS. Causes and clinical manifestations of lymphatic failure. Clin Dermatol. Sep-Oct 1995;13(5):459-71. [Medline].

  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].

Keywords

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

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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