Breast cancer is often detected by a lump, but for less than 5 percent of breast cancer cases, Paget’s disease of the nipple, indicated by a red, inflamed breast area, may be an often overlooked sign of breast cancer.

The cancer, also known as inflammatory breast cancer (IBC), is often mistaken for an infection.

Some women will unsuccessfully try antibiotics to cure it, only to find out it’s Paget’s — a sign of breast cancer.

Symptoms, in some cases, include the formation of a lump underneath the infected area, according to the National Cancer Institute. In 2005 alone, there were 11,000 cases of Paget’s disease of the nipple, which should not be confused with Paget's disease of the bone, which is cited by the NCI as a metabolic bone disease.

The causes, much like most cases of cancer, are still unknown, according to David Sampson, spokesman for the American Cancer Society. Factors like aging, family or personal history of breast cancer, radiation exposure, excess weight, exposure to estrogen and race may be factors. However, women with none of these symptoms have also been diagnosed with the disease.

“I don't know if anyone would venture a guess as to the cause of the disease or risk factors associated with it, but at least we can say it's rare and has an excellent prognosis if caught early,” Sampson said. “Paget's disease starts in the breast ducts and spreads to the skin of the nipple. It may also spread to the areola," which is the round, colored skin surrounding the nipple."

Sampson added, "The skin of the nipple usually appears crusted, scaly and red, with areas of itching, oozing, burning or bleeding. Using the fingertips, a lump may be detected within the breast.”

Signs and Treatment

Paget’s is sometimes difficult to treat and the survival rate is low compared to other cancers, particularly if it has advanced and was overlooked for some time. Most patients who acquire Paget’s are older than 50. However, people as young as age 20 have been diagnosed with the condition.

The disease develops when cancer cells move into the lymph vessels in the skin and block fluid drainage, creating redness and swelling.

As Paget’s disease forms, a patch of scaly, hardened skin may form on the nipple and may spread around the areola (the dark circle around the nipple) and may ooze. Rare forms have formed in both breasts.

Some of the early signs of Paget’s include:

— Redness

— Itching

— Tingling

— A burning sensation

— Skin thickening

— A flattened or inverted nipple

Paget’s may be associated with ductal carcinoma in situ (DCIS) or with infiltrating ductal carcinoma, said Sampson. He added that there also have been cases of Paget’s in men as well.

“Because the male breast is much smaller than the female breast, all male breast cancers start relatively close to the nipple, so spreading to the nipple is more likely,” he said.

Surgical options to treat Paget’s are similar to most cases of breast cancer and include a simple mastectomy, which is removal of the breast but not the lymph nodes in the armpit; skin sparing mastectomy, where the surgeon makes an incision around the nipple and areola and removes underlying breast tissue; modified radical mastectomy, where the breast, underlying chest wall muscle and some of the underarm lymph nodes are removed — an option if invasive breast cancer has occurred; and a lumpectomy, where the diseased part alone is removed.

In about half of patients with Paget’s, according to the NCI, there is a lump or mass felt during an examination, which can signal that the disease has advanced, and surgery may be required. Chemotherapy can also be used in most cases if the disease is caught in its early stages.

Of the current reported cases, according to Sampson, there have been some survival rates, but more research must be done to properly diagnose and treat the rare disease.

“If no lump can be felt and the biopsy shows DCIS but no invasive cancer, the prognosis, outlook for survival, is generally very good,” Sampson said.