There may or may not be a lump in the breast, and there may be redness, oozing and crusting, and a sore that does not heal.The symptoms usually affect the nipple and then spread to the areola and then the breast. It is common for the symptoms to wax and wane. Most women do not visit the doctor because they assume Pagets disease to be minor contact dermatitis or eczema. A lump or skin irritation that does not seem to heal for over a month indicates that attention by a specialist is needed. Pathophysiology
Pagets disease of the breast is characterised by Paget cells. Paget cells are large cells with clear cytoplasm (clear halo) and eccentric, hyperchromic nuclei found throughout the epidermis.There is some controversy as to whether these cells arise from the ductal system of the breast, or whether these cells are a result of in situ malignant transformation.According to the migratory theory, ductal carcinoma in situ cells migrate into the lactiferous sinuses and the nipple skin. Cancer cells disrupt the normal epithelial barrier and extracellular fluid accumulates on the surface of the skin, resulting in the crusting of the areola skin. Diagnosis
Recommended tests are a mammogram and a biopsy to confirm the diagnosis, and cytopathology may also be helpful. Pagets disease is difficult to diagnose due to its resemblance to dermatitis and eczema; even in patients after ductal carcinoma in situ surgery. Eczema tends to affect the areola first, and then the nipple, whereas Pagets spreads from the nipple.
The disorder should be differentiated from several other conditions, especially centrotemporal spikes without seizures, centrotemporal spikes with local brain pathology, central spikes in Rett syndrome and fragile X syndrome, malignant Rolandic epilepsy, temporal lobe epilepsy and Landau-Kleffner syndrome. Treatment
Given the benign nature of the condition and the low seizure frequency, treatment is often unnecessary. If treatment is warranted or preferred by the child and his or her family, antiepileptic drugs can usually control the seizures easily. Carbamazepine is the most frequently used first-line drug, but many other antiepileptic drugs, including valproate, phenytoin, gabapentin, levetiracetam and sultiame have been found effective as well. Bedtime dosing is advised by some. Treatment can be short and drugs can almost certainly be discontinued after two years without seizures and with normal EEG findings, perhaps even earlier. Parental education about Rolandic epilepsy is the cornerstone of correct management. The traumatizing, sometimes long-lasting effect on parents is significant.It is unclear if there are any benefits to clobazam over other seizure medications. Prognosis
The prognosis for Rolandic seizures is invariably excellent, with probably less than 2% risk of developing absence seizures and less often GTCS in adult life. Remission usually occurs within 2–4 years from onset and before the age of 16 years. The total number of seizures is low, the majority of patients having fewer than 10 seizures; 10–20% have just a single seizure. About 10–20% may have frequent seizures, but these also remit with age.