Long term lymphedema can present with a significant number of skin or dermal complications.
Most of these are types of skin growths and plaques directly related to decreased vascular and or lymphatic flow.
While most of these are benign, it is still important for the treating physician to understand the necessity of having these growths biopsied to determine whether or not they are malignant.
One of these complication is the Lymphangiomatous papules. Clinically this is classified as a lymphatic tumor of the soft tissue. Benign appearing lymphangiomatous papules and plaques are the lymphatic counterpart of telangiectasis. These result from acquired permanent dilation of lymphatic capillaries that have appeared in areas of the skin affected by obstruction or destruction of the lymphatics. It is conjecture that they result from interference in the drainage of lymphatic vessels secondary to radiotherapy or surgery.
Also, benign lymphangiomatous papules and plaques may also appear in the skin of elderly patients without evidence of primary lymphatic injury.
There are numerous recorded incidents of these papules presenting with patients who have never received radiotherapy or surgery. It is my opinion that this may be a more common dermal complication involving lymphedema then is noted or realized. I believe the key word term is lymphatic damage, irregardless of “how” the lymphatics were damaged.
Under low magnification, the lesions appear as relatively well-circumscribed capillary proliferation centered in the dermis, without extension into the subcutaneous fat. The epidermis is usually spared. Most lesions show irregularl dilated lymphatic spaces that branch and anastomose within the superficial dermis. The vascular spaces, devoid of content are lined by a discontinuous single layer of endothelial cells with flattened nuclei. Commonly, adjacent vascular channels lie “back-to-back,” separated only by a thin layer of endothelial cells. Multiple papillary projections, covered by a single layer of endothelium, project into he lumina of the dilated lymphatic. The stroma consists of fibrillary collagen rich with spindle, or stellate, fibroblasts. Nodular infiltrates of lymphocytes with germinal centers are occasionally present in the vicinity of the dilated vascular channels. Rarely, vascular proliferation are poorly circumscribed and focally intermingled with irregular jagged vascular spaces that may permeate the entire dermis. Endothelial cels line the latter inconspicuously. Irregular slit-like vascular spaces may be seen between collagen bundles of the dermis, together with tufts of endothelial cells that protrude into the lumina of the newly formed vessels.
The endothelial cells that line the vascular spaces express immunoreactivity for CD31, but they do so only focally or not at all for CD34. Although a minority of newly formed vessels show an attenuated muscle layer, external to the endothelial cells, which has on occasion immunoreactivity for a-smooth muscle actin antibody, this marker is usually non-reactive. Reactivity for Ki-67 is negative among the endothelial cell nuclei. The immunohistochemical profile substantiates the lymphatic nature of these newly formed vessels.
Some dermal vascular responses to irridation, such as benign lymphangioendothelioma or an atypical angiomatous proliferation, may mimic the hisopathologic appearance of the patch stage of Kaposi’s Sarcoma or even a well-differentiated angiosarcoma.(1)
Perhaps, the most common treatment of this growth would be simple surgical removal. However, due to the susceptibility towards infections that lymphadema patients have; it may be safer to leave the growth and remove it only if it becomes too large or begins to present difficulties for the patient’s mobility or well-being.
Mine have only been removed for the two mentioned reasons above. Otherwise, they have actually not presented much of a problem to me.
There are many other skin complications associated with lymphedema. They are listed below with direct links to information articles of each one.
(1)Pathology of Vascular Skin Lesions – Omar P. Sangueza, MD and Luis Requena, MD
Pathologe. 2002 Mar
Mentzel T, Kutzner H. email@example.com
Tumours of lymphatic vessels comprise only a small group in the heterogeneous spectrum of vascular neoplasms of skin and soft tissues. However, this discrepancy between haemangiomas/angiosarcomas and lymphangiomas/lymphangiosarcomas probably represents the present inability to reliably differentiate between lymphatic and capillary vascular endothelium. Histologically, neoplastic lymphatic vessels tend to be lined by endothelial cells with plumper and more prominent, matchstick-like nuclei (in contrast to vascular spaces in haemangiomas that are lined by flat or epithelioid endothelial cells), often show variations in the thickness of the vessel walls and are not completely surrounded by actin-positive (myo)pericytes. Endothelial cells in lymphatic neoplasms tend to be negative for CD34 or stain only focally positive for this marker and an expression of lymphatic markers as vascular endothelial growth factor-C receptor (VEGRF-3), podoplanin and M2A oncofetal antigen has been reported most recently. In addition to “traditional” lymphatic neoplasms including lymphangioma circumscriptum, cavernous lymphangioma/cystic hygroma, benign lymphangioendothelioma, lymphangiomatosis and the rare lymphangiosarcoma, histological and immunohistochemical features of a group of vascular neoplasms with a hobnail cytomorphology (hobnail haemangioma, retiform haemangioendothelioma, papillary intralymphatic angioendothelioma, benign lymphangiomatous papule following radiotherapy) suggest that these lesions also belong to the spectrum of tumours of lymphatic vessels.
Bodet D; Rodríguez-Cano L; Bartralot R; Mollet J; Medina A; Heras C; Bassas P; Huguet P; García-Patos V Department of Dermatology, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Spain.
Benign lymphangiomatous papules of the skin are considered reactive lymphatic proliferations either caused by disruption of the lymphatic flow or tissue damage produced by operation or radiation therapy. We report a 72-year-old woman with umbilical papules and vesicle-like lesions that led to the diagnosis of a large ovarian fibroma. Histologic study revealed dilated lymphatic spaces manifesting an anastomosing and branched pattern in the papillary and reticular dermis dissecting collagen bundles. The vessels were lined by plump endothelial cells with foci of intravascular papillary endothelial cell hyperplasia. After the ovarian fibroma was removed by laparotomy, umbilical lesions almost disappeared, leaving small flesh-colored papules. A periumbilical dermatosis may herald certain intra-abdominal diseases including those of neoplastic derivation. A heightened awareness of this association may lead to an early diagnosis with a potential for improved patient outcome.
Benign lymphangiomatous papules have not been previously described in association with an untreated tumor, without previous operation or radiotherapy. This case advocates for disruption of the lymphatic drainage as the probable pathogenetic mechanism.
Diaz-Cascajo C; Borghi S; Weyers W; Retzlaff H; Requena L; Metze D Centre for Dermatopathology, Freiburg, Germany. firstname.lastname@example.org
AIMS: The aim of this report is to summarize the clinicopathological findings of five cases of a pseudosarcomatous vascular proliferation of the skin at the site of radiotherapy following surgery for carcinoma of the breast and carcinoma of the endometrium. To our knowledge, only five cases of this presumably rare pseudomalignant proliferation have been reported previously.
METHODS AND RESULTS: All patients were females ranging in age from 44 to 70 years. The lesions appeared as solitary or multiple papules or vesicles localized to the field of radiation. Microscopically, they were composed of vascular spaces that exhibited atypical features without qualifying for a diagnosis of angiosarcoma.
CONCLUSIONS: Our cases provide additional evidence supporting the benign nature of this atypical vascular proliferation, not recurring, never developing metastases and being cured readily by local excision. Clinical, histopathological and ultrastructural findings suggest a lymphatic origin. Whether these lesions represent a neoplastic or a reactive condition secondary to radiotherapy is unclear. The name 'benign lymphangiomatous papules of the skin following radiotherapy' is proposed.
Volume 52, Issue 5, Pages 912-913 (May 2005
Kyle Wagamon, MD, Richard E. Ranchoff, MD, Arlene S. Rosenberg, MD, Christine Jaworsky, MD
Benign lymphangiomatous papules of the skin are benign lymphatic proliferations that may arise in the skin after operation or radiation therapy. We report a case of benign lymphangiomatous papules of the skin that occurred in a patient 4 years after undergoing radiation therapy to the chest for adenocarcinoma of the breast.
Am J Surg Pathol. 2002 Mar Requena L, Kutzner H, Mentzel T, Durán R, Rodríguez-Peralto JL. Department of Dermatology, Fundación Jiménez Díaz, Universidad Autónoma, Madrid, Spain.email@example.com
Several types of cutaneous vascular proliferations have been described in areas of irradiated skin, including both benign lesions, such as benign lymphangiomatous papules, atypical vascular lesions, or benign lymphangioendothelioma, and malignant neoplasms such as high-grade angiosarcomas. This report describes the clinicopathologic features of 15 cases of different types of benign cutaneous vascular proliferations arisen within irradiated skin. All patients were female ranging in age from 33 to 72 years, and they had received postoperative external radiotherapy for treatment of breast carcinoma (14 cases) or ovarian carcinoma (one case).
In those cases in which the latency interval period between radiotherapy and the development of the vascular lesion was known from the clinical records, the latency interval period elapsed between radiotherapy and diagnosis of the vascular lesion ranged from 3 to 20 years. The most common clinical presentation of the cutaneous lesions consisted of papules, small vesicles, or erythematous plaques on the irradiated field. Histopathologically, most lesions consisted of irregular dilated vascular spaces, with a branching and anastomosing pattern, thin walls, and lymphatic appearance involving the superficial dermis. A discontinuous single layer of endothelial cells with flattened nuclei lined these vascular channels, and numerous small stromal papillary formations also lined by endothelial cells projected into the lumina of the dilated lymphatic vessels.
These cases were classified as benign lymphangiomatous papules or plaques. Two cases showed different histopathologic findings because they consisted of poorly circumscribed and focally infiltrating irregular jagged vascular spaces involving the entire dermis and lined by inconspicuous endothelial cells. In some areas these irregular slit-like vascular spaces dissected collagen bundles of the dermis. These cases were classified as atypical vascular proliferations mimicking benign lymphangioendothelioma or patch-stage Kaposi's sarcoma. All cases showed similar immunohistochemical findings and the endothelial cells lining the vascular spaces expressed immunoreactivity for CD31, but they stained only focally positive for CD34 or were negative for this marker. Immunohistochemical investigations for alpha-smooth muscle actin failed to demonstrate a complete peripheral ring of actin-positive pericytes in most of the neoformed vascular structures.
This immunohistochemical profile also supported the lymphatic nature of these vascular proliferations developed in irradiated skin. Although some of these lesions may mimic histopathologically patch-stage Kaposi's sarcoma or well-differentiated angiosarcoma, the follow-up of the patients of this series demonstrated that the vascular proliferations arisen in irradiated skin invariably showed a benign biologic behavior
T. Martin-Gonzales, A Sanz-Trelles, J. del Boz, and E. Samaniego
Comment of Article – Letter to the Editor
Lymphangiomas are tumors that normally appear at birth. They are formed from dilated lymph vessels that may extend to the subcutaneous cellular tissue.
A number of casues of acquired lymphgiomas such as radiotherapy and surgery have been reported. The area irridated during radiotherapy may develop benign vascular proliferations such as acquired progressive lymphangiomas or malignant processes such as high-grade angiosarcoma, even when low doses of radiation are used. Within what are considered acquired lymphangiomatous lesions, benign lymphangiomatous papules after radiotherapy have specific characteristics.
Our patient was a 54 yeard old woman with a history of stage T2 N1b M0 infiltrating ductal carcinoma in the right breast, diagnosed in 1998, and treated by tumorectomy, chemotherapy, hormon therapy, and external radiotherapy of the entire chest wall and right breast at a dose 50Gy. She attended our clinic for the evaluation of progressive asymptomatic lesions on the irridated skin of the right breast.
The lesions had appeared approximately 1 year earlier (6 years after receiving radiotherapy).