To the Editor:
Cutaneous vascular lesions are unusual, but are well-documented complications after radiotherapy. Vascular proliferations arisen in areas of previously irradiated skin comprise a wide spectrum of lesions.1 Benign lymphangiomatous papules and plaques are the commonest, but atypical vascular proliferations or even angiosarcomas (AS) may also appear.2 All these vascular lesions emerge within previously irradiated area of skin and appear after several years.3 The morphologic spectrum of these lesions is variable, ranging from small papular lesions characterized by dilated vessels within the upper dermis to large ecchymotic plaques of true AS.1 To date, several case series and reports have been published concerning vascular proliferations in irradiated skin but none of the cases reported so far, has presented with evanescent lesions.
A 62-year-old woman, who had been diagnosed with breast cancer 8 years earlier and treated with conservative surgery followed by chemotherapy and radiotherapy, developed cutaneous lesions which persisted for some days and then disappeared with no residual signs. A week before consulting, she had developed another similar rash. The lesions were multiple reddish papules or small plaques with well-defined borders and were slightly edematous. They were slightly indurated upon palpation, did not exhibit tenderness, and were located in the previously irradiated area, especially around the nipple. One of the lesions, the largest one, was 1.5 cm in diameter and was biopsied (Fig. 1A). The lesions were asymptomatic and the patient reported to have similar rashes during the previous year, in which similar lesions appeared in the same location and persisted for several days until complete spontaneous resolution. One week after the biopsy had been taken, the patient was reevaluated and at this time the lesions had disappeared. A wait-and-see strategy was adopted (Fig. 1B). One year later, the patient continued with periodic rashes, with no symptoms, and no additional lesions have emerged.
The skin biopsy (Fig. 2) revealed a cutaneous vascular proliferation with irregular dilated vessels mainly located in the upper dermis. At scanning magnification the lesion exhibited a wedge-shaped pattern. A layer of endothelial cells demarcated the vascular vessels and their lumen appeared to be empty. In some areas, vascular channels were built “back to back,” with 2 vascular spaces separated only by a thin layer of endothelial cells. The stroma was mainly composed by fibrillary collagen and a slight inflammatory infiltrate was also observed. The immunohistochemical profile revealed immunoreactivity for CD31 and D2-40, this being typical of lymphatic vessels. Upon correlation with the clinical data, a diagnosis of benign vascular postradiotherapy proliferation was made. However, it was striking and confusing that the lesions disappeared without treatment in a few days and that the patient referred to have been developing rashes of the same kind on other occasions.
Vascular proliferations with a benign clinical outcome have been recognized in areas of previously irradiated skin2,4 but evanescence does not seem to be a feature of such lesions, because there is no information about this issue in the literature. Despite the waxing of the lesions, a very slight erythematous background persisted in the whole of the breast of our patient. This waxing and waning of the vascular lesions might be an indicator of severe underlying vascular neoformation (reflecting a different functional state), probably covering the entire breast.
Clinically and pathologically the lesions of our patient are in keeping with the so-called benign lymphangiomatous papules, which have been recognized as the commonest vascular lesions appearing in previously irradiated skin.2 Some authors have suggested other terms for this condition, such us “lymphangioma circunscriptum”5,6 although others consider this to be an inadequate term.7 Benign lymphangiomatous papules (BLAP) appear as permanent dilatations of lymphatic vessels. It has been suggested that these lesions appear in areas of skin affected by the obstruction or destruction of lymphatic drainage, possibly resulting from the injury induced by radiotherapy in these structures.2 The waxing/waning of the lesions in our patient could be explained by admitting that the impairment in lymphatic drainage induced by radiotherapy might initially have a transitory effect until the development of true persistent lesions.
The appearance of lymphatic lesions in previously irradiated skin should be understood as a biologic continuum. In general, lymphatics increase in the female breast after radiotherapy about 2-fold (over a 1-year period). Although this process is reversible, in some cases lymphatic hyperplasia evolves into BLAP. The lesion may stop at this point or continue toward atypical vascular lesions (AVL), this being considered either a precursor or an early stage of AS. In some cases the lesion evolve into florid AS. Currently, clinicians should be aware that it is difficult to define clear-cut borders between lymphatic hyperplasia, BLAP, AVL, and AS. Therefore, these patients should be followed carefully, possibly with multiple biopsies, at different times and at different sites.
The skin of the breast and chest is the area most commonly affected by vascular lesions after radiotherapy.2 All cases described to date have affected women, especially in the sixth decade. They normally appear earlier than true AS. Clinically, BLAP appears as multiple, small, persistent, translucent, vesicles, or papules. Histologically, the vesicles and papules consist of dilated lymphatic vessels, has been described mainly located within papillary dermis. The lumina appear to be empty, and in some cases the presence of intravascular papillary projections has been described.2,4
It is highly questionable whether the lesions in the patient reported here should be called “benign” because lesions such as the one described are well known to recur quite often, and ultimately they may also occasionally progress to AS. Ki-67 was negative in our patient. This has been recognized to be highly expressed in malignant AVLs, primary breast AS, and postradiation AS. Benign AVLs do not express high levels of Ki-67.8 These findings suggest a benign behavior of these lymphatic lesions9 but we are not able to predict their future evolution. This highlights the importance of following up our patient.
This work expands the concept of benign vascular proliferations in previously irradiated skin because we describe a patient with waxing-waning lesions, which is to our knowledge the first report described to date.
Javier Cañueto, MD
Mónica Roncero, MD
Pablo Unamuno, MD, PhD
Department of Dermatology, University Hospital of Salamanca, Paseo San Vicente, Salamanca, Spain
Ángel Santos-Briz, MD, PhD
Department of Pathology, University Hospital of Salamanca, Paseo San Vicente, Salamanca, Spain
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