(Malignant papillary dermatitis; Paget’s disease of the nipple)
Definition – A rarely malignant disease of the nipple and areola, at first characterized by an eczematous condition, later terminating in carcinoma of the whole mammary gland.
Symptoms – This epitheliomatous condition was first described by Paget in 1874 and occurs almost exclusively in women between the ages of forty and sixty, attacking the areola of the right breast in three quarters of the cases. The early stage presents a sharply defined, raw, intensely red, granular-looking patch from which exudes an abundant viscid secretion. The affected surface may be covered with scales or later with crusts and presents burning or itching sensations similar to eczema. From a small lesion less than a half inch in diameter, peripheral extension takes place until all of the areola and often some of the outlying skin is invaded. After a period varying from a few months to years, a button-like infiltration may be felt beneath the skin of the surface lesion which may be readily appreciated by pinching up the patch with the thumb and fingers. Following this induration, perhaps years later, the nipple becomes more and more retracted and nodular growths in the skin and mammary gland develop and ulcerate, followed by the usual cancerous cachexia. Cases of this disease have been reported as occurring on the penis, scrotum, male nipples and other parts but the process in these cases seems identical with the ordinary superficial epithelioma.
Fig. 187 – Paget’s disease of the left nipple of two years’ duration. Slight discharge, burning sensations, marked induration and some retraction of the nipple are present. Cured by Conium 3x internally and 3 per cent. fuchsin ointment externally.
Etiology and Pathology – It is now generally believed that this disease is malignant from the start, although it was formerly held that it was a carcinoma developing upon a keratosis or an eczema. Fissures and persistent irritation of the nipple are predisposing factors. Pathological findings are the same as those met with in the superficial and deep cancers, and by these investigations the malignant nature of the growth is established from its earliest stage.
Diagnosis – The sharp line of demarcation of the patch, its location, the age of the patient, the early raw granular appearance of the lesions and the later button-like infiltration, as well as the course of the disease, should readily establish a diagnosis.
Prognosis and Treatment – A cure may be possible if the condition is recognized before mammary cancer develops but later the course is like that of scirrhus of the breast, in other words fatal. In the earlier stages when doubt exists as to the nature of the disease, the ordinary palliative and soothing applications used in eczema may be needed. At a little later period, fuchsin, 2 to 5 grains to the ounce of lanolin and rose water has achieved good results in a number of reported cases, including one of my own. The X-rays have likewise been of benefit, used alone, preceding an operation or postoperatively. However, radical surgical removal of the breast is the best and should be the first choice in treatment.