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]
Etiology
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[4] ; irradiation alone for various malignancies[5] ; metastatic lymph node obstruction; and various scarring processes, such as infections, keloids, scleroderma, and scrofuloderma.[6] Acquired cutaneous lymphangiectasia has been described due to cervical cancer therapy.[7] Conjunctival lymphangiectasia may represent a biomarker of severe systemic disease in Ser77Tyr hereditary transthyretin amyloidosis.{re38}
Lymphangiectases have been described on 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 or other pelvic cancers.[8, 9, 10, 11] Pelvic lymphatic obstruction may produce acquired vulvar lymphangiomas.[12] Scrotal lymphangiectasia may be evident following scrofuloderma.[13]
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.[14]
Epidemiology
Frequency
The true incidence of lymphangiectasia is not known. Although lymphangiectasia has been reported in the literature with increased frequency in the past two decades, the disease remains rare.
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.
Prognosis
Lymphangiectasia has a good prognosis because most conditions respond well to treatment modalities.
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. Cutaneous angiosarcoma has also been reported in massive localized lymphedema in morbidly obese patients.[15]
Patient Education
Patients should be reassured. Lymphangiectases pose no potential for malignant transformation.
For patient education resources, see the Women's Health Center and Cancer and Tumors Center, as well as Mastectomy and Breast Cancer.
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, chronic lymphedema, or scleroderma.[16, 17] Sometimes, they resolve in weeks without any treatment.[18]
Rarely, lymphangiectasias may occur in pregnancy and spontaneously regress with childbirth.[19] Acquired lymphangiectasia of the glans may also occur after circumcision.[20]
Physical Examination
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 (see the image below). Some lymphangiectasia lesions may become pedunculated with a hyperkeratotic verrucous surface mimicking a wart.[21]
View Image | Lymphangiectasia. Courtesy of DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/doctors/lesions/images/lymphangiectasia1.jpg). |
Conjunctival lymphangiectasis may be evident as intermittent conjunctival swelling and dilated conjunctival vessels on ocular examination.[22]
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.[23]
Lymphangiectasia may also be evident on the penis. Benign transient lymphangiectasis of the penis may be evident.[24] Acquired lymphangiectasia of the glans may occur after circumcision.[20]
Complications
Lymphangiectases are often complicated by pain, copious fluid drainage, and recurrent attacks of cellulitis.
Other Tests
Immunohistochemistry studies are 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
With regard to 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.
Medical Care
Topical sirolimus 0.1% for cutaneous microcystic lymphatic malformations in children and adults is now the subject of a clinical trial since it has been reported as effective in a few patients.[32]
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.
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. 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.
Surgical Care
Many surgical treatment modalities have been advocated in the care of lymphangiectases; these modalities include electrodesiccation, laser therapy,[10] sclerotherapy,[33] cryotherapy, and surgical excision.[21, 34] In a small case series, liquid nitrogen cryotherapy was an effective treatment for localized conjunctival lymphangiectasia.[35] Successful treatment of conjunctival lymphangiectasia has been described using a high-frequency radiowave electrosurgical device.[36] Daily compression through bandaging or hosiery, in accessible areas, has yielded acceptable results. Although no consensus exists concerning therapy for lymphangiectasia, vulvar lymphangiectasia may be effectively handled with carbon dioxide laser therapy.[37] Acquired balanic lymphangiectasia after circumcision was successfully treated with a 2940-nm erbium-doped yttrium aluminium garnet laser.[38]
Author
Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Pathology, Professor of Pediatrics, Professor of Medicine, Rutgers New Jersey Medical School
Disclosure: Nothing to disclose.
Coauthor(s)
Geover Fernandez, MD, FAAD, Staff Physician, Department of Dermatology, Rutgers New Jersey Medical School
Disclosure: Nothing to disclose.
Specialty Editors
David F Butler, MD, Former Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic
Disclosure: Nothing to disclose.
Rosalie Elenitsas, MD, Herman Beerman Professor of Dermatology, University of Pennsylvania School of Medicine; Director, Penn Cutaneous Pathology Services, Department of Dermatology, University of Pennsylvania Health System
Disclosure: Received royalty from Lippincott Williams Wilkins for textbook editor.
Chief Editor
Dirk M Elston, MD, Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine
Disclosure: Nothing to disclose.
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