Scrofula is a condition in which the bacteria that causes tuberculosis causes symptoms outside the lungs. This usually takes the form of inflamed and irritated lymph nodes in the neck.

Doctors also call scrofula “cervical tuberculous lymphadenitis”:

  • Cervical refers to the neck.
  • Lymphadenitis refers to inflammation in the lymph nodes, which are part of the body’s immune system.

Scrofula is the most common form of tuberculosis infection that occurs outside the lungs.

Historically, scrofula was called the “king’s evil.” Until the 18th century, doctors thought the only way to cure the disease was to be touched by a member of a royal family.

Fortunately, doctors know much more now about how to identify, diagnose, and treat this condition.

Pictures of scrofula

What are the symptoms?

Scrofula most commonly causes swelling and lesions on the side of the neck. This is usually a swollen lymph node or nodes that may feel like a small, round nodule. The nodule usually isn’t tender or warm to the touch. The lesion may start to get bigger and may even drain pus or other fluid after several weeks.

In addition to these symptoms, a person with scrofula may experience:

Scrofula is less common in industrialized nations where tuberculosis isn’t a common infectious disease. Scrofula represents of tuberculosis cases doctors diagnose in the United States. Tuberculosis in nonindustrialized nations.

What causes this?

Mycobacterium tuberculosis, a bacterium, is the most common cause of scrofula in adults. However, Mycobacterium avium intracellulare can also cause scrofula in a minority of cases.

In children, nontuberculosis bacteria causes are more common. Children can contract the condition from putting contaminated items in their mouths.

Risk factors

People who are immunocompromised are at greater risk for scrofula. Scrofula accounts for an estimated of all cases of tuberculosis in immunocompromised people in the United States.

For someone who is immunocompromised due either to an underlying condition or medication, their body doesn’t have as many immune system cells, especially T cells, to fight off infections. As a result, they’re more vulnerable to get the condition.

Those with HIV who are on antiretroviral therapies tend to experience greater inflammatory responses to tuberculosis bacteria.

How is it diagnosed?

If a doctor suspects the tuberculosis bacteria could be causing your neck mass, they’ll often perform a test known as a purified protein derivative (PPD) test. This test involves injecting a small amount of PPD just underneath the skin.

If you have tuberculosis bacteria present in your body, you’ll experience an induration (a raised area of the skin that is several millimeters in size). However, because other bacteria can cause scrofula, this test isn’t 100 percent definitive.

Doctors usually diagnose scrofula by taking a biopsy of the fluid and tissue inside the inflamed area or areas around the neck. The most common approach is a fine-needle biopsy. This involves carefully taking measures not to spread the bacteria to surrounding areas.

A doctor may first order some imaging scans, such as an X-ray, to determine how involved the mass or masses are in the neck and if they look like other scrofula cases. Sometimes, initially, a doctor can mistakenly identify scrofula as a cancerous neck mass.

There aren’t any specific blood tests to diagnose scrofula. However, your doctor may still order blood tests, such as cat-scratch titers and HIV testing, to rule out other conditions.

Treatment options

Scrofula is a serious infection and can require treatment over the course of several months. A doctor typically prescribes antibiotics for six months or longer. For the first two months of treatment, people often take multiple antibiotics, such as:

  • isoniazid
  • rifampin
  • ethambutol

After this time, they’ll take isoniazid and rifampin for roughly four additional months.

During the course of therapy, it isn’t unusual for the lymph nodes to get larger or for new inflamed lymph nodes to appear. This is known as a “paradoxical upgrading reaction.” It’s important to stick with the treatment even if this happens.

Sometimes doctors may also prescribe oral steroids, which can help to reduce inflammation in the scrofula lesions.

A doctor might recommend surgically removing the neck mass or masses after treatment with antibiotics. However, the mass isn’t usually treated until the bacteria are no longer present. Otherwise, the bacteria can cause a fistula, which is a tunneled hole between the infected lymph node and the body. This effect can cause further severe symptoms.

Possible complications

of those who have scrofula also have tuberculosis in their lungs. It’s possible that scrofula can spread beyond the neck and affect other areas of the body.

Also, a person can experience a chronic, draining open wound from the neck. This open wound can allow other types of bacteria into the body, which can lead to further serious infections.

What’s the outlook?

With antibiotic treatment, cure rates for scrofula are excellent, at about . If you suspect you could have tuberculosis or you have symptoms of scrofula, see your doctor for a tuberculosis skin test. These are also available at many city and county health departments as a fast and low-cost way to diagnose tuberculosis.