Letter to the Editor
Extramammary Paget's disease of the scalp: examination by in vivo and ex vivo reflectance confocal microscopy
Article first published online: 25 JUN 2013
© 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd
Skin Research and Technology
Volume 20, Issue 1, pages 124–126, February 2014
How to Cite
Debarbieux, S., Dalle, S., Depaepe, L., Jeanniot, P. Y., Poulalhon, N. and Thomas, L. (2014), Extramammary Paget's disease of the scalp: examination by in vivo and ex vivo reflectance confocal microscopy. Skin Research and Technology, 20: 124–126. doi: 10.1111/srt.12087
- Issue published online: 10 JAN 2014
- Article first published online: 25 JUN 2013
- University Lyon 1
- Hospices Civils de Lyon
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.
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. . 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).
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. Interestingly, Haas and Hauptmann reported a case of alopecia neoplastica corresponding to an epidermotropic metastasis of a previously treated breast carcinoma. 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.
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
The authors state no conflict of interest.
- 4A case of extramammary Paget's disease on the scalp. Rinshohifuka 1994; 48: 359., , et al.