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.
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.
Frequency
United States
The true incidence of this disease 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.
Sex
No sexual predominance has been reported.
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 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 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.
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 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 therapy; irradiation alone for various malignancies; metastatic lymph node obstruction; and various scarring processes, such as infections, keloids, scleroderma, and scrofuloderma.
- 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.
- Acquired lymphangiomas have been reported in the genital region of elderly patients without evidence of lymphatic obstruction.
More on Lymphangiectasia |
 Overview: Lymphangiectasia |
| Differential Diagnoses & Workup: Lymphangiectasia |
| Treatment & Medication: Lymphangiectasia |
| Follow-up: Lymphangiectasia |
| References |
| Next Page » |
References
Ahmed DD, Waldorf JC, Randle HW. Cutaneous lymphangiectasis: treatment with sclerotherapy. Plast Reconstr Surg. Feb 1998;101(2):434-6. [Medline].
Ambrojo P, Cogolludo EF, Aguilar A, et al. 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].
Celis AV, Gaughf CN, Sangueza OP, Gourdin FW. Acquired lymphangiectasis. South Med J. Jan 1999;92(1):69-72. [Medline].
Di Leonardo M, Jacoby RA. Acquired cutaneous lymphangiectasias secondary to scarring from scrofuloderma. J Am Acad Dermatol. Apr 1986;14(4):688-90. [Medline].
Garcia-Doval I, de la Torre C, Losada A, et al. Acquired cutaneous lymphangiectasia in a patient with cirrhotic ascites. J Eur Acad Dermatol Venereol. Sep 1999;13(2):109-12. [Medline].
Handfield-Jones SE, Prendiville WJ, Norman S. Vulval lymphangiectasia. Genitourin Med. Oct 1989;65(5):335-7. [Medline].
Harwood CA, Mortimer PS. Causes and clinical manifestations of lymphatic failure. Clin Dermatol. Sep-Oct 1995;13(5):459-71. [Medline].
Harwood CA, Mortimer PS. Acquired vulval lymphangiomata mimicking genital warts. Br J Dermatol. Sep 1993;129(3):334-6. [Medline].
Hunt SJ, Santa Cruz DJ. Vascular tumors of the skin: a selective review. Semin Diagn Pathol. Aug 2004;21(3):166-218. [Medline].
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].
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].
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].
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].
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].
Moon SE, Youn JI, Lee YS. Acquired cutaneous lymphangiectasia. Br J Dermatol. Aug 1993;129(2):193-5. [Medline].
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].
Noel AA, Gloviczki P, Bender CE, et al. Treatment of symptomatic primary chylous disorders. J Vasc Surg. Nov 2001;34(5):785-91. [Medline].
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].
Pena JM, Ford MJ. Cutaneous lymphangiectases associated with severe photoaging and topical corticosteroid application. J Cutan Pathol. Apr 1996;23(2):175-81. [Medline].
Schwab RA, McCollough ML. Acquired vulvar lymphangiomas: a sequela of radiation therapy. Cutis. Mar 2001;67(3):239-40. [Medline].
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].
Ziv R, Schewach-Millet M, Trau H. Lymphangiectasia. A complication of thoracotomy for bronchial carcinoid. Int J Dermatol. Mar 1988;27(2):123. [Medline].
del Pozo J, Martínez W, Yebra-Pimentel MT, Fonseca E. Lymphangiectatic variant of pilomatricoma. J Eur Acad Dermatol Venereol. Sep 2004;18(5):575-6. [Medline].
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].
Further Reading
Keywords
acquired lymphangioma, acquired lymphangiectasia, secondary lymphangioma, lymphangioma circumscriptum
