Depigmented genital extramammary Paget’s disease: A possible histogenetic link to Toker’s clear cells and clear cell papulosis
Version of Record online: 7 JUL 2008
Journal of Cutaneous Pathology
Volume 28, Issue 2, pages 105–108, February 2001
How to Cite
Chen, Y.-H., Wong, T.-W. and Lee, J. Y.-Y. (2001), Depigmented genital extramammary Paget’s disease: A possible histogenetic link to Toker’s clear cells and clear cell papulosis. Journal of Cutaneous Pathology, 28: 105–108. doi: 10.1034/j.1600-0560.2001.280208.x
- Issue online: 7 JUL 2008
- Version of Record online: 7 JUL 2008
- Accepted October 25, 2000
Background: The histogenesis of extramammary Paget’s disease (EMPD) is still controversial. Benign pagetoid cells of the nipple first described by Toker and the similar clear cells found in white maculopapules of clear cell papulosis (CCP) have been proposed to be potential precursor cells giving rise to EMPD and primary intraepidermal Paget’s disease in the nipple. The observation of a rare case of depigmented EMPD provided us with a chance to examine further the interesting Toker’s clear cell/CCP hypothesis.
Methods: We performed pathologic studies, including Fontana-Masson stain and immunostaining for AE1/AE3 and S100P, on a new case of depigmented EMPD manifesting a 4×3 cm hypopigmented-depigmented patch on the root of the penis.
Results: The lesion showed extensive intraepithelial proliferation of atypical pagetoid cells with markedly reduced epidermal melaninization but nearly normal numbers of melanocytes. The tumor cells were strongly positive for AE1/AE3 by immunostaining. Some tumor cells displayed tadpole-like morphology resembling the pagetoid cells of CCP. Such morphology was not observed in two random examples of non-depigmented genital EMPD.
Conclusions: The findings of tadpole-shaped pagetoid cells and depigmentation in the present case suggest that depigmented EMPD may be histogenetically related to CCP. Depigmented EMPD should be considered in the differential diagnosis of vitiligo, depigmented mycosis fungoides and lichen sclerosus located along the milk line.