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# Dubin-Johnson syndrome

A benign inherited condition in which the liver poorly excretes conjugated bilirubin into bile; the liver looks black.

Updated 2026-11-01

## Structures involved

Tap one to see what it is and open it in 3D.

- [Liver](https://durgical.com/en/anatomy/liver/)
- [Gallbladder](https://durgical.com/en/anatomy/gallbladder/)
- [Common hepatic duct](https://durgical.com/en/anatomy/common-hepatic-duct/)

## In biochemistry

- [Heme breakdown and bilirubin: Step 4 · MRP2 (ABCC2), canalicular transporter](https://durgical.com/en/biochemistry/pathways/heme-breakdown-and-bilirubin/#paso-4)
- [Bilirubin](https://durgical.com/en/biochemistry/molecules/bilirubin/)

## In physiology

- [Bile and the enterohepatic circulation](https://durgical.com/en/physiology/bile-and-the-enterohepatic-circulation/)

## Why it happens

- [Pigments in tissue: lipofuscin, hemosiderin, melanin, bilirubin, carbon](https://durgical.com/en/general-pathology/pigments-in-tissue-lipofuscin-hemosiderin-melanin-bilirubin-carbon/)

## What it is

Chronic conjugated hyperbilirubinemia from failure of the canalicular transporter MRP2 (ABCC2 gene), with dark pigment in hepatocytes.

## Who gets it

Rare; its highest frequency (about 1 in 1300) has been described among Jews of Iranian and Moroccan origin; isolated cases worldwide.

## Causes

- Autosomal recessive inheritance: ABCC2 variants

## Risk factors

- Pregnancy and oral contraceptives (increase jaundice)

## Pathophysiology

Bilirubin is conjugated normally, but without MRP2 it cannot leave into the canaliculus and refluxes into the blood. A melanin-like pigment (adrenaline derivatives) collects in lysosomes and darkens the liver. Coproporphyrin excretion also changes: total urinary coproporphyrin is normal but over 80 % is isomer I.

## Symptoms and signs

- Mild, intermittent jaundice
- Sometimes dark urine or right upper quadrant discomfort
- Many have no symptoms

## Red flags

- Itching, pale stools or raised cholestatic enzymes (suggest another cause)

## Diagnosis

**Clinical**
Mild conjugated jaundice in a young adult with otherwise normal [liver](https://durgical.com/en/anatomy/liver/) tests.

**Tests**
High direct bilirubin with normal alkaline phosphatase and transaminases; urinary coproporphyrins over 80 % isomer I; ABCC2 gene testing.

**Imaging**
Hepatobiliary scintigraphy shows prolonged liver visualisation with delayed [gallbladder](https://durgical.com/en/anatomy/gallbladder/) filling; ultrasound rules out obstruction.

## Treatment

**Conservative**
No treatment is needed; its benign nature is explained

**Follow-up**
Watching jaundice during pregnancy or with hormonal contraceptives

## Complications

- None serious; possible interactions with drugs excreted by MRP2

## Prognosis

Excellent.

## Test yourself

Think of the answer, then tap the question to see it.

## Which transporter fails in Dubin-Johnson syndrome?

MRP2 (ABCC2), which pumps conjugated bilirubin into bile.

## Which gross finding is typical of the liver in Dubin-Johnson?

A black liver from melanin-like pigment in lysosomes.

## How do urinary coproporphyrins distinguish Dubin-Johnson from Rotor?

In Dubin-Johnson the total is normal with over 80 % isomer I; in Rotor the total is high.

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## Related conditions

- [Rotor syndrome](https://durgical.com/en/conditions/rotor-syndrome/)

Educational information, not medical advice. For any symptom, see a health professional.

Sources: [Dubin-Johnson syndrome: MedlinePlus Genetics](https://medlineplus.gov/genetics/condition/dubin-johnson-syndrome/) (MedlinePlus, US National Library of Medicine, 2026); [ABCC2 gene: MedlinePlus Genetics](https://medlineplus.gov/genetics/gene/abcc2/) (MedlinePlus, US National Library of Medicine, 2026); [Bilirubin blood test: MedlinePlus Medical Encyclopedia](https://medlineplus.gov/ency/article/003479.htm) (MedlinePlus, US National Library of Medicine, 2026).

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Source: https://durgical.com/en/conditions/dubin-johnson-syndrome/
License: CC BY 4.0 (https://creativecommons.org/licenses/by/4.0/). Cite as: DurgiPedia (durgical.com), https://durgical.com/en/conditions/dubin-johnson-syndrome/