An Overview of Cullen Sign

Cullen sign (sometimes called Cullen's sign) is a term used to describe a discoloration around the navel. It appears in the fatty tissue as swelling, bruising, and an unusual color. Cullen sign may be seen in patients with certain health conditions, such as:

• Pancreatitis, inflammation of the pancreas
  
• Ectopic pregnancy
• Liver problems
• Thyroid cancer
• Other sources of internal bleeding in the abdomen

This condition often coexists with Grey Turner’s sign. Grey Turner's sign appears as bruising of the abdominal walls or flanks.

Cullen sign is relatively rare. It’s seen in less than 1% of ectopic pregnancies, and in 1% to 3% of pancreatitis patients. When it does appear, it's considered a marker of serious health problems.

This article looks at the symptoms, causes, and diagnosis of Cullen sign. It also reviews how the diseases associated with this symptom are treated.

Cullen Sign Symptoms

Cullen sign appears as severe discoloration, bruising, and swelling around the navel. The color of the bruise varies based on severity. In moderate cases, it may be yellow or green. In severe cases, it may be purple. Some patients may feel pain and discomfort in the area.

Causes of Cullen Sign

Cullen sign happens when blood and/or pancreatic enzymes pool in tissues just beneath the skin. A range of conditions might cause this, including:

Acute Pancreatitis

The pancreas is an organ that produces digestive enzymes and hormones. Pancreatitis happens when digestive enzymes become active before they are released into the small intestine. These enzymes damage the pancreas, which can cause pain, internal bleeding, and cysts. Acute cases form quickly and can even be fatal if left untreated.

In cases of acute pancreatitis, Cullen sign tends to show up 24 to 72 hours after the onset of the condition. Studies have found that up to 37% of pancreatitis cases that have this marker end up being fatal.

Pancreatic/Abdominal Trauma

Injury to the pancreas or abdominal region due to an accident can cause internal bleeding that results in Cullen sign.

Ectopic Pregnancy

An ectopic pregnancy happens when a fertilized egg attaches to a location outside the uterus, most commonly in the fallopian tubes. These pregnancies aren't viable, meaning they can't come to term. If left untreated, they can cause dangerous internal bleeding.

Ruptured Abdominal Aorta

The abdominal aorta is a major artery that moves blood from the heart to the abdomen. When it ruptures, it can cause severe pain in the area as well as low back pain. It can also lead to Cullen sign.

Ruptured Spleen

The spleen is an organ located under the left rib cage. A rupture of the spleen causes blood to pool in the abdomen and is considered a medical emergency.

Ovarian Cysts

Ovarian cysts are fluid-filled sacs in the ovaries. In most cases, this very common condition does not have symptoms. Sometimes, though, a cyst can grow so large it needs to be removed. When this happens, Cullen sign may appear.

Thyroid Cancer

The thyroid is a butterfly-shaped gland in the throat. Cullen sign can be one of many symptoms of cancer in this gland. In many cases, however, thyroid cancer has no symptoms.

Esophageal Cancer

Esophageal cancer affects the esophagus, which connects the throat to the stomach. Cullen sign can appear when the cancer metastasizes or spreads throughout the body. The prognosis in these cases is very poor. 

Non-Hodgkin Lymphoma

Non-Hodgkin lymphoma is a cancer of the white blood cells. This can cause a range of symptoms including swelling of the lymph nodes in the throat. It can also damage the spleen, which can lead to Cullen sign.

Percutaneous Liver Biopsy

This test involves extracting a tissue sample from the liver using a special kind of needle. It is usually done when doctors suspect cancer. In rare cases, this test can cause excessive bleeding that leads to Cullen sign.

Amoebic Liver Abscess

Entamoeba histolytica is an amoeba that can infect the tissues of the liver, causing a buildup of pus. This kind of infection is more common in areas with poor sanitation and crowded conditions. It can lead to a range of health problems and can appear as Cullen sign.

Strangulated Umbilical Hernia

The umbilical cord connects the mother and fetus during pregnancy. The cord is cut after birth and heals up to form the belly button. In around 20% of cases, though, it can take much longer to heal or not heal at all. The bleeding that happens in the abdominal area can lead to Cullen sign.

Diagnosis

When doctors see Cullen sign, it indicates they need to diagnose the underlying cause. Tests used to diagnose the many possible causes vary widely and may include blood tests and imaging tests.

Treatment

Treating Cullen sign means treating the disorder or disease that's causing it. Pancreatitis, for example, can be treated with:

• Directed fasting
• Fluid replacement via IV
• Medication for pain
• Surgery of the pancreas or gallbladder

Ectopic pregnancy can be treated with drugs that prevent cell growth, laparoscopic surgery, or other medical procedures. Cancers often require surgery, chemotherapy, or radiation treatment. 

Summary

Cullen sign is a discoloration and swelling around the belly button. It usually appears in conjunction with a serious underlying medical condition.

Pancreatitis, ectopic pregnancy, and certain cancers are a few of the conditions associated with Cullen sign.

Diagnosis and treatment of Cullen sign focus on identifying the underlying cause.

A Word From Verywell

Many of the underlying issues that cause Cullen sign are severe and require immediate medical attention.

If you’re seeing signs of this condition, talk to your healthcare provider right away. The sooner they can find the cause, the better. In some cases, early diagnosis can mean the difference between life and death.