Cullen sign is a medical finding that signals significant internal bleeding within the abdomen or retroperitoneal space. This superficial manifestation is also known as periumbilical ecchymosis. The discoloration represents blood products that have tracked from a deeper injury to the skin’s surface. The presence of this sign is a physical clue that directs medical professionals to search for life-threatening intra-abdominal pathology requiring immediate evaluation.
Appearance and Location of the Sign
Cullen sign is characterized by bruising and swelling specifically located around the umbilicus (belly button). The ecchymosis can appear as a bluish, purplish, or sometimes greenish-yellow patch. These color variations reflect the stage of red blood cell breakdown in the subcutaneous tissue, with newer bleeding appearing darker and older bleeding shifting toward yellow.
This discoloration occurs because blood from an internal hemorrhage migrates through the abdominal fascial planes to the anterior abdominal wall. The blood products pool in the loose connective tissue surrounding the navel, making this area the visible endpoint. The sign often takes 24 to 48 hours to develop after the initial internal event as the blood travels to the surface.
The Primary Cause: Hemorrhagic Pancreatitis
The condition most commonly associated with Cullen sign is acute hemorrhagic pancreatitis, a severe inflammation of the pancreas. In this pathology, powerful digestive enzymes become prematurely activated, beginning to digest the pancreatic tissue itself (autodigestion). This process can erode blood vessels within and around the organ.
This enzymatic erosion leads to significant bleeding and the leakage of hemorrhagic fluid into the retroperitoneal space. The blood and fluid track along anatomical pathways, moving superiorly along the falciform ligament. This fluid eventually reaches the subcutaneous fat near the umbilicus, producing the visible bruising.
The presence of Cullen sign in a patient with pancreatitis is an ominous finding, suggesting the disease has progressed to a severe, necrotizing stage with associated hemorrhage. This is a relatively rare occurrence, appearing in only one to three percent of acute pancreatitis cases. However, when it occurs, it is linked to a much higher mortality rate, sometimes reaching 37 percent.
Associated Conditions
While acute hemorrhagic pancreatitis is the most frequent cause, Cullen sign can result from any condition that causes substantial bleeding within the abdominal or retroperitoneal cavities.
Other Causes of Cullen Sign
- Ruptured ectopic pregnancy, which occurs when a fertilized egg implants outside the uterus, causing rapid intraperitoneal bleeding.
- Blunt abdominal trauma resulting in internal organ damage and hemorrhage.
- A leaking or ruptured abdominal aortic aneurysm (AAA), a highly lethal source of internal bleeding.
- Splenic rupture.
- Rectus sheath hematoma (bleeding within the sheath of the rectus abdominis muscle).
- Certain coagulation disorders that predispose a person to spontaneous hemorrhage.
Urgency and Initial Medical Response
The appearance of Cullen sign indicates a massive, life-threatening internal hemorrhage requiring immediate emergency medical intervention. Medical staff quickly initiate fluid resuscitation to stabilize the patient’s blood pressure and address hypovolemic shock caused by blood loss. This involves administering intravenous fluids and blood products to restore circulating volume.
Diagnostic testing is performed simultaneously to pinpoint the source of the bleeding. Blood work includes a complete blood count and tests for pancreatic enzymes (amylase and lipase). A pregnancy test is routinely performed for women of childbearing age. Imaging studies, such as a computed tomography (CT) scan, are performed urgently to visualize the hemorrhage and identify the damaged organ. Treatment is targeted directly at the underlying cause, often involving emergency surgery to control the hemorrhage.