Paget's disease

Paget's disease most commonly occurs in the breast, where most patients have an underlying ductal carcinoma. It has also been reported from extramammary sites, most commonly the vulva but also the perianal region, perineum, groin, pubic area, scrotum, penis, thigh, buttock, axilla and eyelids. There is usually no underlying carcinoma. The histogenesis of extramammary Paget's disease is controversial. The mucin profile of vulval Paget's disease most closely matches that of the Bartholin's gland and of clear cell papulosis (described in children and characterised by multiple small maculopapules composed of clear cells resembling the Toker cells of the nipple and thought to represent ectopic eccrine cells1)

Immunohistochemistry

Both cytokeratin 7 and MUC1 may be useful in the identification of Paget's cells.

References

1 Kuan, S. F., Montag, A. G., Hart, J., Krausz, T., Recant, W. Differential Expression of Mucin Genes in Mammary and Extramammary Paget's Disease. Am J Surg Pathol 2001;25:1469-1477

2 Liegl B, Horn LC,Moinfar F. Androgen receptors are frequently expressed in mammary and extramammary Paget's disease. Mod Pathol 2005; 18:1283-8

This page last revised 1.4.2006.

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