Dubin-Johnson Syndrome: Causes, Symptoms, and Treatment
Medically reviewed by Harsha Moole, MD, Internal Medicine. Last reviewed: October 23, 2026.
What kind of doctor treats Dubin-Johnson Syndrome?
A hepatologist (a liver doctor) or a gastroenterologist (a digestive system doctor) usually cares for Dubin-Johnson syndrome, an inherited cause of mild jaundice. A genetic counselor can also help families understand the chance of passing it on. A direct primary care or concierge doctor can be a first stop for an evaluation and can refer you to a specialist when needed.
What is Dubin-Johnson syndrome?
Dubin-Johnson syndrome is a disorder passed down through families.
It is a very rare genetic disorder.
It causes jaundice, a yellowing of the skin and the whites of the eyes. The jaundice is mild and lasts throughout life.
In most people, certain deposits build up in the liver but do not seem to harm how it works. The deposits make the liver look black on imaging.
Symptoms
Mild jaundice is most often the only symptom. It may not appear until puberty or adulthood.
Some people also have weakness, mild belly pain, nausea, or vomiting.
Rarely, jaundice starts soon after birth. These babies typically also have an enlarged liver and a severely reduced ability to make and release bile, a digestive fluid.
As these children get older, the liver problems go away. They usually have no related health problems later in life.
The jaundice may get worse with alcohol, birth control pills, infection, pregnancy, fasting or dehydration, and fatigue.
Complications are unusual. They may include belly pain and severe jaundice.
Causes and who gets it
Dubin-Johnson syndrome is caused by changes in a gene called ABCC2.
This gene makes a protein that moves bilirubin out of liver cells and into bile. Bilirubin is a yellow-orange substance made when old red blood cells break down.
With the changed gene, bilirubin builds up in the body. This causes the yellow color of the skin and eyes.
The condition is inherited in an autosomal recessive pattern. This means a child must get a copy of the changed gene from both parents.
The parents each carry one changed copy, but they typically show no signs of the condition.
It appears to be most common in Iranian and Moroccan Jews living in Israel. It appears to be less common in other groups.
How doctors diagnose it
These tests can help diagnose the syndrome:
- A liver biopsy, which checks a small sample of liver tissue.
- A blood test for liver enzyme levels.
- A blood test for serum bilirubin.
- Urine tests for coproporphyrin levels.
Treatment
No specific treatment is required.
The outlook is very positive. The syndrome generally does not shorten a person's lifespan.
If you have a family history of the syndrome, genetic counseling may help if you plan to have children.
When to see a doctor
Contact your health care provider if:
- Your jaundice is severe.
- Your jaundice gets worse over time.
- You also have belly pain or other symptoms. These may be a sign that another disorder is causing the jaundice.
How a direct primary care or concierge doctor fits in
A direct primary care doctor charges a flat monthly fee instead of billing insurance, while a concierge doctor usually charges more and often works alongside insurance. A direct primary care doctor can order liver blood tests, follow your jaundice over time, and refer you to a liver specialist or genetic counselor.
Common questions
Is Dubin-Johnson syndrome dangerous?
Usually not, because the outlook is very positive. The syndrome generally does not shorten a person's lifespan, and complications are unusual.
Does Dubin-Johnson syndrome need treatment?
No, no specific treatment is required. Contact your provider if the jaundice is severe or gets worse over time.
Is Dubin-Johnson syndrome inherited?
Yes, it is a disorder passed down through families. A child must get a copy of the changed gene from both parents, and genetic counseling may help families who plan to have children.
When does the jaundice start?
In most people, jaundice starts in the teen years or early adulthood.
Sources
Medical Disclaimer
This content is for general educational and informational purposes only and is not medical advice. It is not a substitute for professional medical advice, diagnosis, or treatment, and reading it does not create a doctor-patient relationship. Always seek the advice of your physician or another qualified health provider with any questions about a medical condition, and never disregard or delay seeking professional advice because of something you read here. If you think you may have a medical emergency, call 911 (or your local emergency number) immediately.
