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Cullen sign

GastroenterologyGastrointestinalPancreasAbdomenReproductiveIntegumentary

Summary

Cullen sign is periumbilical ecchymosis (bluish discoloration) caused by retroperitoneal or intraperitoneal hemorrhage tracking to the umbilicus. Classically associated with hemorrhagic (necrotizing) acute pancreatitis, but also seen in ruptured ectopic pregnancy and other causes of hemoperitoneum. It signals severe disease and is a poor prognostic sign.

Detail

**Definition:** Superficial edema and bruising (blue-black/purple discoloration) around the umbilicus.

**Pathophysiology:** In severe acute pancreatitis, activated pancreatic enzymes (trypsin, elastase, phospholipase A2) digest retroperitoneal and peripancreatic tissue and vessel walls, causing hemorrhage. Blood and exudate dissect along fascial planes, tracking through the gastrohepatic and falciform ligaments to the subcutaneous tissue of the umbilicus. Hemoglobin breakdown produces the discoloration. The sign typically appears 24-72 hours after onset (sometimes later).

**Associated sign:** Grey Turner sign is flank ecchymosis from retroperitoneal blood tracking along the lateral abdominal wall. Both signs indicate hemorrhagic pancreatitis and are present in only about 1-3% of cases. Neither is specific to pancreatitis.

**Differential causes:** - Acute hemorrhagic/necrotizing pancreatitis (classic) - Ruptured ectopic pregnancy (the original description by Thomas Cullen, 1918) - Other hemoperitoneum: ruptured abdominal aortic aneurysm, splenic or hepatic rupture or trauma - Retroperitoneal hemorrhage, including from anticoagulation - Strangulated bowel or other abdominal catastrophe

**Clinical significance:** - Marks severe disease with a higher risk of necrosis, organ failure, and mortality. - Workup and management for pancreatitis: lipase (more specific) and amylase, CT abdomen with contrast (to assess necrosis, usually after 72 hours), aggressive IV fluids (lactated Ringer's), analgesia, early enteral nutrition, and ICU-level monitoring if needed. - If the patient is a woman of childbearing age with abdominal pain and shock, get a β-hCG and consider ruptured ectopic pregnancy. - Complications of severe pancreatitis include pancreatic necrosis, pseudocyst, abscess, ARDS, hypocalcemia, DIC, and acute kidney injury. - Ranson criteria and BISAP are prognostic scoring systems.

**High-yield pearls for boards:** - Cullen = Umbilicus. Grey Turner = flank (Turner = "turn" to the side). - Hypocalcemia in pancreatitis results from saponification of fat (calcium binds free fatty acids) and is a poor prognostic marker. - Common etiologies of pancreatitis: gallstones and alcohol (I GET SMASHED mnemonic for the full list). - Fox sign is inguinal ligament ecchymosis, a related finding. - Cullen sign is also described in ruptured ectopic pregnancy, which is a common Step 2 vignette.

Sources

  • First Aid for the USMLE Step 1
  • Harrison's Principles of Internal Medicine, chapter on Acute and Chronic Pancreatitis
  • Robbins and Cotran Pathologic Basis of Disease, Pancreas
  • UWorld Step 1/Step 2 CK Question Bank
  • Sleisenger and Fordtran's Gastrointestinal and Liver Disease
  • Cullen TS. A new sign in ruptured extrauterine pregnancy. Am J Obstet Dis Women Child. 1918

Reviewed by Medusa editorial — medical student review. Information here is for study reference only and is not medical advice. Spotted an error? Let us know.