Paget’s disease of the nipple

Paget’s disease of the nipple typically presents as a persistent abnormality of the nipple–areola complex, often resembling eczema or chronic dermatitis. The nipple or areola may become erythematous, hyperpigmented, crusted and thickened. It is hardened or nodular, with surface scaling, fissuring or ulceration. Nipple inversion, discharge, bleeding or serous exudation may occur.

Patients often report itching, burning, pain, tingling or soreness. Because the early appearances may be subtle and mimic benign inflammatory disease, persistent unilateral nipple–areolar change should prompt assessment for underlying breast malignancy.

It is a rare complication of breast cancer, present in 0.5 – 5% of breast carcinomas, and is more common in post-menopausal females. Most cases are associated with underlying ductal carcinoma (or ductal carcinoma in situ), and some with lobular carcinoma. Research is still ongoing into the apparent formation of the above cancers in the breast, and its transformation into physical presentation at the nipple.

Investigations include triple assessment of the breast by clinical examination, ultrasound and fine needle aspiration for cytology. Skin scrapings of the affected tissue are also useful, particularly in early or superficial disease.

Treatment involves surgery, radiotherapy and chemotherapy for advanced cases.  

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History of Paget’s disease of the nipple

1856 – Alfred-Armand-Louis-Marie Velpeau (1795-1867) described chronic destructive dermatoses of the nipple–areola complex. He did not define the condition as a distinct clinicopathological entity and did not clearly connect the nipple disease with underlying mammary carcinoma.

1874 – Sir James Paget (1814-1899) published On diseases of the mammary areola preceding cancer of the mammary gland in the St Bartholomew’s Hospital Reports. He reported about fifteen women, aged forty to sixty or older, in whom a chronic eruption of the nipple and areola preceded the development of scirrhous carcinoma of the breast.

Paget then gave the clinical description that remains recognisable today:

The patients were all women, various in age from 40 to 60 or more years, having in common nothing remarkable but their disease. In all of them the disease begins as an eruption of the nipple and areola. In the majority it had the appearance of a florid, intensely red, raw surface, very fine and granular, as if nearly the whole thickness of the epidermis were removed; like the surface of very acute diffuse eczema, or like that of an acute balanitis. From such a surface, on the whole or greater part of the nipple and areola, there was always copious, clear, yellowish, viscid exudation. The sensations were commonly tingling, itching, and burning, but the malady was never attended by disturbance of the general health.

Paget recognised the temporal association with breast cancer:

But it has happened in every case which I have been able to watch, cancer of the mammary gland has followed within at the most two years, and usually within one year… The formation of cancer has not in any case taken place first in the diseased part of the skin. It has always been in the substance of the mammary gland, beneath or not far from the diseased skin, and always of a clear interval of apparently healthy tissue

He also noted that, in his cases, the cancer appeared to arise in the breast rather than in the diseased skin itself:

A second series of hard cancers, deviating from the usual forms, consists of cases in which the nipple and skin or other tissues of the mammary gland are peculiarly affected.

1880 – Scottish surgeon Robert Wardrop Forrest (1840-1919) reported the first male case consistent with mammary Paget disease. His patient, a seventy-two-year-old man, had progressive nipple disease with crusting, erythema, local pain and itching, nipple destruction and underlying induration.

This case, which now presents the appearance of an ordinary cancer of the breast, derives its interest chiefly from its history as given by the patient, which shows it to be allied to the cases of disease of the mammary areola preceding cancer of the mammary gland first described by Sir James Paget. It is interesting also from the patient being a male, the cases hitherto recorded having all been females.

Forrest, 1880

1889 – Henry Radcliffe Crocker (1845-1909) extended the concept in with the first reliable description of extramammary Paget disease. This established that the Paget pattern was not confined to the nipple–areola complex, although mammary and extramammary Paget disease have different clinical settings and pathogenesis.

Paget was not the first person to observe chronic nipple disease, but recognised its repeated association with mammary carcinoma. Later microscopy corrected Paget’s theory that the nipple eruption was a benign dermatitis preceding cancer, but was an intraepidermal malignant process associated with underlying ductal carcinoma.

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Associated Persons

• Alfred-Armand-Louis-Marie Velpeau (1795-1867)
• James Paget (1814–1899)
• Robert Wardrop Forrest (1840-1919)
• Henry Radcliffe Crocker (1845-1909)

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References

Original articles

• Paget J. On diseases of the mammary areola preceding cancer of the mammary gland. St. Bartholomew Hospital Reports, London, 1874, 10: 87-89.
• Velpeau A. Maladies du sein chez la femme In: Leçons orales de clinique chirurgicale faites à l’Hôpital de la charité. 1840: 68-96 [Tranlsation: A treatise on the diseases of the breast and mammary region, 1840]
• Forrest RW. Case of cancer of the mamma in the male, preceded by so-called eczema of the mammary areola, “Paget’s disease of the nipple.” Glasgow Medical Journal. 1880;14(12):457–460.
• Crocker HR. Paget’s disease affecting the scrotum and penis. Transactions of the Pathological Society of London. 1889;40:187–191.

Review articles

• Velpeau AALM. Traité des maladies du sein et de la région mammaire. 1854 [Translation: A treatise on the diseases of the breast and mammary region] 1856
• Sandoval-Leon AC, Drews-Elger K, Gomez-Fernandez CR, Yepes MM, Lippman ME. Paget’s disease of the nipple. Breast Cancer Res Treat. 2013 Aug;141(1):1-12.
• Tjalma WA, Bosteels J, Thiessen F, Smets K. Rewriting the history of Paget’s disease: From Arderne’s medieval case to Crocker’s first extramammary description (1370-1889). Eur J Obstet Gynecol Reprod Biol. 2026 Jun;322:115082.