Lymphangiectasia Information

What is Lymphangiectasia?

Lymphangiectasia

Last Updated: November 8, 2005

Synonyms and related keywords: acquired lymphangioma, acquired lymphangiectasia, secondary lymphangioma, lymphangioma circumscriptum

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

Geover Fernandez, MD, is a member of the following medical societies: Alpha Omega Alpha

Editor(s): James Fulton, Jr, MD, PhD, Medical Director, Fulton Skin Institute; David F Butler, MD, Professor, Texas A&M University College of Medicine; Director, Division of Dermatology, Scott and White Clinic; Rosalie Elenitsas, MD, Associate Professor of Dermatology, University of Pennsylvania School of Medicine; Director, Penn Cutaneous Pathology Services, Department of Dermatology, University of Pennsylvania Health System; Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University; and Dirk M Elston, MD, Teaching Faculty, Department of Dermatology, Geisinger Medical Center

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:

In the US: 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.

Differentials

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

Other Problems to be Considered:

A lymphangiectatic variant of pilomatricoma has been described (del Pozo, 2004).

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.

Treatments

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, sclerotherapy, cryotherapy, and surgical excision.

Daily compression through bandaging or hosiery, in accessible areas, has yielded acceptable results.

Followup

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 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

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