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A 54-year-old male patient was referred for a 3 years lasting 6 cm erythematous patch of the posterior vertex that had progressively enlarged (Fig.1). There was no personal or familial history of neoplasia. Physical exam was otherwise unremarkable. Pathological study of a punch biopsy sample revealed an intra-epidermal proliferation of large atypical clear cells, isolated or in nests. Immunostainings were CK7 positive and CK20 negative, suggestive of an extramammary Paget's disease (EMPD). A work-up was performed to exclude an underlying malignancy: cervical ultrasound scan, gastroscopy, urine cytopathology, and PET TDM were found negative.

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Figure 1. (a) Clinical aspect of the lesion. Left blue circle: positive area under RCM (5 mm from the clinical margin); anterior blue circle: negative area under RCM (10 mm from the clinical margin). (b) In vivo confocal microscopy performed in the center of the lesion: numerous large roundish atypical dark cells (arrows). (c–e) Ex vivo examination with Vivascope® 3000: the surgical slice is placed flat between 2 pieces of translucent food wraps and covered with vaseline oil.

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In vivo reflectance confocal microscopy was performed. We observed intra-epidermal large dark isolated or nested cells, with few of them exhibiting a target appearance (Fig.1). RCM examination of the 5 mm margin outside the clinical margins revealed atypical cells whereas no atypical cells were seen on the 10 mm margin (Fig.1). These findings were in concordance with the pathological data and typical features of Paget's disease as recently reported by Guitera et al. [1]. Surgical excision was therefore indicated with a 15 mm margin and an extemporaneous ex vivo confocal examination was performed on a 2 mm large slice taken off the maximum diameter of the surgical specimen. Since we did not have a confocal microscope dedicated to ex vivo use, we used the Vivascope 3000® (Lucid Inc, Rochester, NY, USA). To this end, the surgical slice was placed flat on a compress between translucent disposable food wraps and covered with vaselin oil (Fig. 2). This allowed to visualize the proliferation of large roundish Paget's cells within the epidermis, isolated or in nests, and to confirm there was no such cell near the limits of the slice (Fig. 2).

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Figure 2. Ex vivo confocal examination and histopathological correlation. (a): Ex vivo confocal aspect in the center of the slice: large atypical roundish cells with a so-called « target appearance » (arrows), with formation of small nests. (c) Histopathological correlation (HES × 20) on the slice examined by RCM (Paget's cells identified by arrows). (b) Ex vivo confocal aspect of the extremity of the slice. Normal epidermis characterized by a regular honeycomb appearance with no Paget's cell. (d) Histopathological correlation (HES × 20) on the slice examined by RCM showing a normal epidermis with no Paget's cell.

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Paget's disease is an intra-epidermal adenocarcinoma that has primarily been described involving the nipple, where it is associated with an underlying breast carcinoma. Later on, it was described on other sites, mainly ano-genitals, where it is primarily intra-epidermal though it can be associated with an internal carcinoma. It has been reported that anogenital Paget's disease CK20+/GCDFP- would be likely to be associated with a regional internal carcinoma with a corresponding immunophenotype[2, 3].

Only 3 cases of extramammary Paget's disease (EMPD) affecting the scalp have been reported in the literature [4-6]; two of them were primarily cutaneous EMPD[4, 5] whereas one was associated with an underlying hidradenocarcinoma[6]. Interestingly, Haas and Hauptmann reported a case of alopecia neoplastica corresponding to an epidermotropic metastasis of a previously treated breast carcinoma[7]. In that case, the patient's history and immunochemistry were favoring the diagnosis. In our case, CK20 was found negative and the work-up was negative.

This case also illustrates an original use of the Vivascope V3000. Indeed, ex vivo examination has never been reported with that device. Although the images are less accurate than those obtained using the fluorescence mode, the quality was sufficient enough to identify the Paget's cells, thanks to their very large size, and confirm that the extremities of the slice examined were in sano. This extemporaneous method is worth evaluating in other cases as an alternative to the use of a dedicated device.

Acknowledgements

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Funding: Work supported in part by a grant from the University Lyon 1 (to LT) and a grant from the Hospices Civils de Lyon (to LT).

Conflicts of interest

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  2. Acknowledgements
  3. Conflicts of interest
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The authors state no conflict of interest.

References

  1. Top of page
  2. Acknowledgements
  3. Conflicts of interest
  4. References