Rosai-Dorfman disease (RDD) is a rare, benign condition that involves an overgrowth of white blood cells called histiocytes. It usually causes swollen lymph glands in your neck. But the overgrowths can also affect your skin and other organs. Treatments include surgery, corticosteroids and targeted therapy. The outlook varies from person to person.
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Rosai-Dorfman disease (RDD) is a rare, noncancerous overgrowth of white blood cells called histiocytes. Usually, the excess cells cause enlarged, swollen lymph nodes in your neck (lymphadenopathy). But they can cause swelling in other lymph nodes, too. These cells can also collect in places like your skin or sinuses. They can involve organs like your breast, heart and bones, among others.
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Histiocytes are an important type of cell in your immune system. They fight germs that may make you sick. But having too many of them can lead to inflammation that damages tissue and causes symptoms.
Another name for RDD is sinus histiocytosis with massive lymphadenopathy.
Rosai-Dorfman affects people differently. The cells may affect one group of lymph nodes or many groups. The histiocytes may collect in parts of your body other than your lymph nodes. These differences help experts classify RDD into types, like:
About 4 out of 10 people with RDD have excess histiocytes in extranodal sites in addition to their lymph nodes.
Symptoms depend on where the histiocytes build up in your body. You may have mild symptoms or even no symptoms if the histiocytes only affect lymph nodes in your neck. More severe symptoms can happen if the histiocytes impact organ function.
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Usually, histiocytes grow in the lymph nodes in your neck. The most common symptom is painless, swollen lumps on both sides of your neck. You may have swelling in other lymph node sites, like your:
Swelling may be your only symptom. Or you may also have:
Rosai-Dorfman disease that affects your skin (CRDD) causes growths that may appear anywhere on your body. They usually grow slowly and can look:
Symptoms of excess histiocytes in an organ vary based on location. RDD that affects your sinuses may cause stuffiness; RDD that affects your eyes may cause double vision; RDD affecting your brain may cause seizures; etc.
Medical experts don’t know what causes Rosai-Dorfman disease. It affects people so differently that it may have many causes. CRDD (the kind that affects your skin) likely has different causes than classic (nodal) RDD.
Researchers have found that about 3 to 5 out of 10 people with RDD share common gene mutations (changes). These mutations can cause cells to grow out of control. Knowing about these mutations has helped experts develop new treatments for RDD.
Many people with RDD also have other conditions. There may be a connection between these conditions and RDD. More research is needed to know for sure.
RDD is associated with:
Rosai-Dorfman disease mainly affects children, teens and young adults. Most diagnoses occur at about age 20. But it can affect people of all ages.
Cutaneous RDD mostly affects people in their 20s, 30s and 40s.
A healthcare provider will ask about your symptoms. They’ll check for signs of RDD, like swollen lymph glands and skin growths. They’ll also ask about your medical history to see if you have (or have previously had) any of the conditions that may happen along with RDD.
You may also need:
You may not need treatment if you don’t have symptoms. Instead, your healthcare provider may monitor your condition.
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Surgery to remove damaged tissue or lymph nodes can treat local disease. “Local” means the histiocytes are only in one place. Sometimes, radiation therapy is an option to treat local RDD. Your healthcare provider may prescribe steroid creams to treat RDD on one area of your skin.
If the histiocytes are spread out in your body, you’ll likely need to see providers who specialize in treating those body parts. They’ll work together to find the best treatment plan for you. Treatments include:
You’ll see your provider regularly, whether you receive treatment or not. If you don’t need treatment, your provider will still monitor you closely so they can provide care ASAP as needed. If you do need therapy, they’ll monitor you to see how it’s working.
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In the meantime, let your provider know if you’re having unexpected symptoms or side effects. They’ll talk you through what to expect so you know what changes count as “not normal.”
For the most part, RDD isn’t usually life-threatening or seriously damaging to organs. It doesn’t impact your life expectancy. Newer treatments, like targeted therapy, have greatly improved the outlook of people living with this condition.
Your prognosis (outlook) depends on various factors. They include how many lymph nodes are affected and where the excess histiocytes are in your body. Your response to treatment also matters.
Sometimes, RDD goes away on its own (spontaneous remission), but the timing can be unpredictable. It may go away in a few months or a few years. Other times, the condition doesn’t improve on its own, or the cells grow back following remission. In general, the fewer lymph nodes affected, the better your prognosis.
No. Rosai-Dorfman is benign. This means it doesn’t spread to other parts of your body, as malignancies (cancer) can. Still, that doesn’t mean that it can’t be serious. The growths involved with RDD can impact organs and cause issues in some people. Your healthcare provider is your best resource for understanding how RDD affects your body.
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Rosai-Dorfman disease (RDD) can be a challenging diagnosis because it’s so rare and affects people differently. There isn’t just one set of guidelines to treat it. This is why it’s very important to talk with your healthcare provider so you understand what to expect from treatment. You may have a mild case of RDD that goes away on its own, or you may need a combination of treatments to manage a more serious disease. Ask your provider how your condition will shape your care plan and likely outcomes.
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Cleveland Clinic’s health articles are based on evidence-backed information and review by medical professionals to ensure accuracy, reliability and up-to-date clinical standards.
Cleveland Clinic’s health articles are based on evidence-backed information and review by medical professionals to ensure accuracy, reliability and up-to-date clinical standards.
Rosai-Dorfman disease is rare — but not to Cleveland Clinic’s expert healthcare providers. Our team has the personalized treatment you need for this blood condition.
