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BACKGROUND: Paget's disease is an intraepidermal adenocarcinoma that is difficult to diagnose clinically as it mimics inflammatory or infectious diseases. As a consequence, it may be clinically misdiagnosed resulting in a delay in appropriate management. Reflectance confocal microscopy allows the visualization of the upper layers of the skin and mucosa at cellular resolution. Paget's disease is characterized histologically by the presence of neoplastic cells scattered throughout all layers of the epidermis in a pattern similar to that also observed in melanoma (and termed Pagetoid spread). OBJECTIVE: In vivo confocal microscopy is an excellent diagnostic tool for detecting Pagetoid spread and for diagnosing melanoma. We therefore hypothesized that it may also assist in the diagnosis of Paget's disease. METHODS: In this study, we describe the confocal features of nine cases of extramammary Paget's disease and one case of mammary one. RESULTS: Large atypical Pagetoid cells were present singly and in clusters in all 10 cases and were readily visualized on ex vivo and in vivo confocal microscopy. The presence of Pagetoid spread and other confocal features, in the appropriate clinical context, is suggestive Paget's disease and should allow distinction from other inflammatory diseases that may appear similar clinically. CONCLUSION: The use of confocal microscopy is likely to facilitate earlier diagnosis of Paget's disease and the instigation of appropriate management with concomitant improvement in clinical outcomes.

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A hitherto unreported variant of mammary Paget's disease (MPD) limited to the areola, leaving the nipple unaffected, has been analyzed by serial sectioning of the whole areola and nipple. This otherwise characteristic MPD proved to be confined to the epidermis. There was no underlying carcinoma. This MPD was associated with a multifocal presence of monomorphic but otherwise similar cells in small collections surrounding the ostia of areolar mammary glands in the clinically unaffected area. This condition was interpreted as hyperplasia of mammary gland-related cells also found in normal nipples (so-called Toker cells). The observations hint at a possible derivation of some cases of mammary and extramammary Paget's disease from such Toker cells.

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An ultrastructural study of the epidermis from eight patients with clinical Paget's disease of the nipple supports the epidermotropic theory. There was no evidence that the Paget's cells originated from keratinocytes. We propose the hypothesis that Paget's cells represent transformed ductal cells, from the subjacent lactiferous ducts of the nipple, which have migrated into the epidermis, either as neoplastic cells or as normal ductal cells with secondary in situ transformation in the epidermis.

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A 72-year-old woman had a 7-year history of a scaly red area on the right breast which had enlarged asymptomatically. A biopsy of the nipple was taken, and, following the finding of Paget's disease, a modified radical mastectomy was carried out. On histological examination of the entire breast specimen by serial sections, no evidence of an intraductal adenocarcinoma was found. Fractionation of Paget cells was performed from the epidermis. Stripped skin was treated with EDTA and trypsin, and epidermal cell suspensions were obtained. They were layered onto discontinuous Percoll gradients and centrifuged. Paget cells fell into three fractions with densities of 1.041, 1.058, and 1.078. Electronmicroscopically, the purity of fractionated cells obtained by this method ranged from 55 to 74% with viabilities of from 70 to 90%. Microscopic analysis of the DNA content in these cells was performed. The DNA histogram was close to the normal ploidy. This may explain why the mammary Paget's disease lesions in this case enlarged rather slowly.

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Os autores relatam dois casos de doença de Paget mamária ocorridos no Hospital Universitário em Brasília, durante o ano de 1989. Em um dos casos, a lesäo era pigmentada, simulando melanoma e no outro, a lesäo era, francamente, eczematosa. É feita uma breve revisäo da literatura

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Twenty-one cases of Paget's disease have been studied using histochemical, ultrastructural, and immunohistochemical methods. Eight of the tumors involved the nipple, and 13 were extramammary (11 vulvar and two anal). The antibodies used were directed against different classes of cytokeratin proteins, epithelial membrane antigen, carcinoembryonic antigen, gross cystic disease fluid protein-15, and S-100 protein. The findings of this study provide conclusive evidence that Paget's cells, regardless of their location, are adenocarcinoma cells. Intracytoplasmic mucin is scanty in Paget's cells within the nipple, but typically plentiful in the extramammary sites where the cells are frequently signet-ring cells. The common mechanism for the evolution of Paget's disease is extension of cells from an underlying carcinoma, but the possibility that some cases, particularly in the vulva, develop from intraepithelial precursors cannot be excluded.

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A case of Paget's disease of the vulva associated with Paget's disease of the nipple is reported. When the patient was 74-year-old, Paget's disease of the vulva was diagnosed; four years later, Paget's disease of the nipple was detected. Paget's disease of the vulva, although recurrent, is devoid of underlying infiltrating carcinoma. Eccrine sweats glands show different type of lesions consistent with pagetoid extension, in situ carcinoma and epithelial papillary proliferation. Ultrastructural study demonstrates dense granules within Paget's cells. A positive Thiery's cytochemical reaction indicates that these granules contain glycoprotein. Paget's disease of the nipple is associated with an intraductal carcinoma (cribriform type). Some feature of intralobular carcinoma is also observed. No foci of infiltration are found. Oestrogen receptors are detected by the histofluorescence technique of Sin Hang Lee. Few receptors are seen within the intraductal neoplastic cells and within the Paget's cells whereas no receptor at all, are observed within the keratinocytes. Several reports emphasized the relationship between vulvar Paget's disease and infiltrating breast carcinoma. Associated vulvar and nipple Paget's disease is rather rare and was just observed in two cases. These two observations were associated with a mammary infiltrating carcinoma whereas in our case, it remains an in situ carcinoma.

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A case of mammary Paget's disease has been studied under electron microscopy. It seems that Paget's cells are coming from the basal layer which is not affected by the malignant proliferation. Paget's cells show some characters of keratinocytes such as rudimentary desmosomes and tonofilaments. But the tumoral tissue is very loose. The Paget's cells are not keratinised; they present numerous vesicles as malignant cells of apocrin mammary carcinoma. There are numerous histogenetic theories of Paget's disease. One of them sounds very interesting: it consists in looking for associations between microscopic mammary Paget's disease and mammary apocrin carcinoma.

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