Inflammatory breast cancer (IBC) is defined more by what it is not than by what it is. This rare, aggressive type of cancer doesn’t fit the description of what we commonly think of as breast cancer.

IBC is highly visible, and women often find it themselves rather than having it detected through screening. Cancer cells block lymph vessels in the skin, making the breast look red, swollen and inflamed. This can also cause nipple distortion and pain. IBC usually doesn’t present as a palpable lump.

Rare but not new, IBC was written about in the early 19th century by Scottish surgeon Sir Charles Bell. Artists have depicted this distinctive presentation in their work, including Michelangelo in his sculpture Night. However, most people have never heard of this type of breast cancer and it is not commonly taught in medical or nursing schools.

Without even a medical coding number, we can only guesstimate the incidence of IBC. Experts think IBC accounts for 1 to 5 percent of all breast cancers diagnosed in the United States, but it may be responsible for about 10 percent of breast cancer deaths. It’s more common among African-American women and, for reasons not fully understood, seems to be more aggressive in these women compared with white women. Men can and do get breast cancer, including IBC, but this is very uncommon.

Most IBC is invasive ductal carcinoma, which means it develops from cells that line the milk ducts and spreads beyond them. Mammograms and ultrasounds often miss it because of the scattered tumor cells. IBC tends to strike women at a younger age, when the breasts are naturally more dense, potentially masking the tumors. It is often initially misdiagnosed as an infection (mastitis) or an injury.

IBC progresses rapidly—often in just weeks or months—and this rapid onset of symptoms is key to diagnosis. About 75 percent of people with IBC are diagnosed at Stage III and the rest at Stage IV, meaning it has spread to distant sites in the body.

The standard of care for IBC is similar to that of locally advanced breast cancer, but mid-treatment scans are done more often to assess the effectiveness of chemotherapy. Half of inflammatory breast cancers are hormone-receptor negative, which means that estrogen does not promote their growth and hormone therapy is ineffective.

After chemotherapy has shrunk the cancer, people with IBC usually undergo a non-skin-sparing mastectomy, which is necessary to remove cancer that has invaded the skin. Radiation is also an important part of trimodal care for the best outcomes for this aggressive disease.

Today, although still not widely known, IBC is getting more attention. Hope is arising from dedicated IBC research and specialists developing new treatment paradigms for this virulent cancer with a short life expectancy.