Aged Woman With Large Lesion on Chest

HISTORY An 85-year-old woman who resides on the dementia unit of a nursing home is seen for routine care. A large lesion on the anterior chest is noted. Her husband, an extremely devoted visitor who is cognitively intact, avers that the lesion has been unchanged since their wedding some 60 years before. Fragile skin, frequent skin tears. Ecchymoses common even after trivial or no trauma. Solar (senile) purpura. Not taking anticoagulants; no known liver disease or coagulopathy. Severely symptomatic gastroesophageal reflux disease, now controlled with metoclopramide and H2 blockade. Chronic urinary retention. PHYSICAL EXAMINATION Pleasant but nonverbal woman who appears stated age. In no distress. Normal vital signs. Long-standing urethral catheter. No sign of irritation at its entry point. Urine in the collection bag clear, not murky. Skin of anterior chest as shown. Early basal cell carcinoma of the face. No prominent purpura on this visit. WHAT’S YOUR DIAGNOSIS? Answer on next page  , ANSWER: CAPILLARY MALFORMATION (PORT-WINE STAIN) Figure 1 – Closer view emphasizes the irregularity not only of shape, but of border and of interface between lesion and adjacent normal skin. Resolving ecchymosis adjoins left lateral edge of the inferior surface. The right nipple is visible at the extreme lower edge of the photograph, and the inframammary skin fold is prominent. This patient’s left breast appears more pendulous in this image as an artifact of positioning. In the same way, the apparent fold of skin superomedial to the right nipple is artifactual. Background skin appears thin, as evidenced by prominence of the vascular network, flat surface, and paucity of markings. A large patch covers much of the anterior chest skin to the left of the midline and overlying part of the sternum. This purple area appears poorly circumscribed, an impression that is heightened the more closely one looks (Figures 1 and 2). Nowhere is there thickening or nodularity in this lesion, nor is it ulcerated. At the edges in particular, a fine pattern of dots seems to make up the lesion and to account for difficulty in determining precisely where the lesion ends and normal skin begins (see Figure 2). The lesion is a so-called port-wine stain, which is actually a congenital capillary malformation. An area of resolving ecchymosis is faintly visible just below the lower left portion of the lesion. Farther inferolaterally, an area of slight darkening represents shadowing by a slight concavity of the body surface. EXPERIMENTS Strictly in the interests of accuracy, the authors attempted to correlate the popular name of this lesion with a replication of the pathogenesis implied by that name: thus, port wine was studied in glass (Figure 3), on the skin both wet (Figure 4) and dry (Figure 5), on tissue paper (see Figure 4), and on a towel (Figures 6 and 7). None of these closely mimicked the patient’s capillary malformation, which confirms that old macroscopic clinical labels and names, however vivid and imaginative and even fanciful, sometimes lacked scientific accuracy. One could of course counter that port wine is no longer as syrupy and therefore discoloring as it used to be. Figure 4 – The same port wine applied to the skin lacks both the color and the texture of the lesion. A tissue employed to blot the excess shows only a disappointing gray-brown tint. IS IT A HEMANGIOMA? For decades or centuries, these lesions have been given the more scientifically proper-sounding names of cavernous or capillary hemangiomas, and a dizzying variety of other names. Yet these, too, are inappropriate: the lesions have no features of benign neoplasia.1  Rather, they are developmental, originating in  the 4th to 10th week of fetal life.1  Their origin may reflect local sparsity of autonomic innervation, leading to vasodilation. Histologically, the endothelium is normal but the capillary spaces are enlarged. The lesions often darken over the years, and can thicken as well, but they tend not to enlarge. Hence the husband’s report of 6 decades without change is fully credible. Nor do these lesions involute as many of the true neoplastic hemangiomas of infancy do. Figure 5 – When port wine dries on the skin, any vestige of resemblance between a true port-wine stain and the eponymous lesion is lost. COULD IT BE MALIGNANT? If we apply the classic ABCD melanoma criteria, this lesion displays multiple features that could aggregately suggest malignancy: asymmetry, irregular borders, variegation of color (though all are shades of red), and diameter far larger than that of a pencil eraser. However, the lesion is not a neoplasm nor is it brownblack, and as it happens is not of melanocytic origin. Nor do the ABCD features predict increased malignant potential in capillary hemangiomas. An experienced dermatologic consultant was completely confident that the lesion was benign; he did not recommend biopsy, even though he was planning to perform biopsy of the facial basal cell lesion. If an area of thickening or nodularity develops in a capillary hemangioma, clinicians may worry about malignant change. However, thickening and the formation of nodules occur commonly in capillary malformations over a lifetime, and neither change portends malignant transformation.2  Despite an awareness of this phenomenon, the senior author at first wondered if the lesion might be a non–HIV-related Kaposi sarcoma.3  He based this hypothesis on the highly irregular border and his own misperception that the tiny vessels at the edge were curlicue-shaped and not dot-like, and on an over-interpretation of the ecchymosis. The latter was traumatic and perhaps related to the patient’s tendency to solar purpura and to her fragile skin. MANAGEMENT IN EVOLUTION Because these lesions (many of which occur on the face4) are of concern primarily from a cosmetic point of view, a plethora of cover-ups and treatments have been devised. The most consistently effective means to lighten the lesions is a pulsed-flash laser with a wavelength of 585 nm. This technique produces significant fading in most persons and near-complete fading in some 15%. Recent work to determine the depth of the affected vessels with a videomicroscope holds hope for more focused treatment with better results.5  Recurrences after excellent fading have prompted the hypothesis that an underlying localized lack of vascular reactivity predisposes the patient to redilate new capillaries in the same area, with reappearance of the same alteration in skin color.6 REFERENCES: 1. Grevelink SV, Mulliken JB. Vascular anomalies. In: Freedberg I, Eisen A, Wolff K, et al, eds. Dermatology in General Medicine. 5th ed. New York: McGraw-Hill Book Company; 1999:1175-1177, 1185-1186. 2. Klapman MH, Yao JF. Thickening and nodules in port-wine stains. J Am Acad Dermatol. 2001;44:300-302. 3. Schneiderman H. Classic (non-HIV-associated) Kaposi’s sarcoma of the feet in the elderly. Consultant. 1993;33(11):99-101. 4. Mills CM, Lanigan SW, Hughes J, et al. Demographic study of port wine stain patients attending a laser clinic: family history, prevalence of naevus anaemicus and results of prior treatment. Clin Exp Dermatol. 1997;22:166-168. 5. Eubanks LE, McBurney EI. Videomicroscopy of port-wine stains: correlation of location and depth of lesion. J Am Acad Dermatol. 2001;44:948-951. 6. Ozluer SM, Barlow RJ. Partial re-emergence of a port-wine stain following successful treatment with flashlamp-pumped dye laser. Clin Exp Dermatol. 2001;26:37-39.