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] and underlying lymphatic filariasis. [14, 15]
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. [16]
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. [17]
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.
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Lymphangiectasia. Courtesy of DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/doctors/lesions/images/lymphangiectasia1.jpg).
