Scrofula

Scrofula

Article
Healthy Lungs
Diseases & Conditions
+3
Contributed byKrish Tangella MD, MBAJan 15, 2019

What are the other Names for this Condition? (Also known as/Synonyms)

  • Cervical Tuberculous Lymphadenitis
  • Lymph Node Tuberculosis
  • Tuberculous Lymphadenitis of Neck Lymph Nodes

What is Scrofula? (Definition/Background Information)

  • The most common form of tuberculosis is pulmonary tuberculosis in which the lungs are primarily affected. Scrofula is tuberculous infection of the lymph nodes in the neck. The condition is also known as Lymph Node Tuberculosis
  • Scrofula usually affects individuals with compromised immune system, such as HIV patients and those taking immunosuppressant medications (for various health reasons)
  • The tuberculous infection spreads to different parts of the body through blood or lymph in immuno-compromised individuals. This may also lead to infection of the lymph nodes, called lymphadenitis. When the size of the lymph nodes increases, it causes erosion of the overlying skin and leads to drainage of purulent material
  • The causative organisms differ in adults and children. Disseminated infection occurs with Mycobacterium tuberculosis (a pathogenic bacterium) in adults and nontuberculous mycobacterium, which includes Mycobacterium scrofulaceum and/or Mycobacterium avium, in children
  • The most important symptom of Scrofula is the presence of a firm, rubbery, neck swelling. The condition may be diagnosed through a fine needle aspiration test of the affected lymph node
  • The treatment may involve both medical and surgical interventions, depending upon the infecting organism. The prognosis of Scrofula is very good with appropriate treatment

Who gets Scrofula? (Age and Sex Distribution)

  • Scrofula is most commonly seen in individuals with poor or compromised immune systems.
  • There is no sexual predilection, both males and females are affected
  • The condition is observed worldwide; generally all racial and ethnic groups have the same amount of risk

What are the Risk Factors for Scrofula? (Predisposing Factors)

An important risk factor for the occurrence of Scrofula includes defects in the cell-mediated immunity, due to various health conditions and causes. The following individuals are at risk of Scrofula:

  • Human immunodeficiency virus infected individuals, AIDS patients
  • Organ transplant individuals treated with immunosuppressant medications
  • Chemotherapy for malignant tumors
  • Congenital immunodeficiency syndromes, which are immunodeficiency conditions present at the time of birth

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Scrofula? (Etiology)

There are many different types of mycobacteria. Scrofula is caused by rod-shaped bacteria such as Mycobacterium tuberculosis, Mycobacterium scrofulaceum, and Mycobacterium avium.

  • Generally, Mycobacterium tuberculosis is the most common cause of Scrofula in adults
  • In children, Scrofula is caused by Mycobacterium scrofulaceum and Mycobacterium avium in a higher frequency
  • Infection in adults occur from Mycobacterium tuberculosis, a pathogenic species responsible for most tuberculosis cases
  • Infections in children occur from nontuberculous mycobacterium (NTM), which is a group of mycobacteria species also referred to as atypical or environmental mycobacteria
  • Mycobacterium scrofulaceum and/or Mycobacterium avium (both NTMs) are the causative agents of Scrofula in children

What are the Signs and Symptoms of Scrofula?

The signs and symptoms of Scrofula depend upon whether it is caused by Mycobacterium tuberculosis or a non-Mycobacterium tuberculosis bacterium.

Mycobacterium tuberculosis infection (in adults) signs and symptoms may include:

  • Fever, chills
  • Firm, rubbery, neck swelling in the front part of the neck
  • Multiple neck swellings (lymph nodes) that are stuck together (matted)
  • Draining fistula from the matted lymph nodes

Non-tuberculous mycobacterium infection (in children) signs and symptoms may include:

  • The individuals may not have any symptoms, such as fever and chills
  • There may be neck swelling similar to M. tuberculosis infection
  • No history of previous TB exposure

How is Scrofula Diagnosed?

The diagnosis of Scrofula includes a complete evaluation of the medical history along with a thorough physical exam. Other diagnostic tests may include:

  • Complete blood count
  • Fine needle aspiration (FNA) of the lymph node, in which the material inside the lymph node is aspirated with the help of a needle and sent to the laboratory for examination under a microscope by the pathologist
  • Tissue culture can help in a definitive diagnosis of Scrofula
  • Biopsy of the lymph node and examination of the material under the microscope by a pathologist
  • Test for HIV (this test is not required for the diagnosis of  scrofula but is done to check the immune status of the patient)
  • MRI or CT scan of the neck, to examine the exact site and extent of the disease
  • Chest X-ray to check for the presence of pulmonary tuberculosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Scrofula?

Possible complications of Scrofula include:

  • Chronic draining fistula of the skin in the neck region
  • Scar formation resulting in cosmetic disfigurement

How is Scrofula Treated?

The treatment for Scrofula depends on the causative organism.

If the condition is caused by Mycobacterium tuberculosis, the treatment may involve the following:

  • Isoniazid, rifampicin, pyrazinamide, ethambutol for 2 months, followed by 2 drugs (usually isoniazid and rifampin) according to the organism sensitivity for another 4 months
  • Baseline liver function tests, kidney function tests, and platelet level tests should be done before starting the treatment, because the drug side effects can affect the liver and kidney function

Surgical management of M. tuberculosis infection:

  • Surgery is reserved for chronic cases not responding to proper medical treatment and for patients with draining fistula
  • Patients should be treated with anti-tuberculosis infection prior to surgery, to prevent dissemination (spread) of the disease

If the condition is caused by non-Mycobacterium tuberculosis (such as Mycobacterium scrofulaceum and Mycobacterium avium), the treatment may involve the following:

  • Clarithromycin is the drug of choice
  • Most of the patients require surgery. The surgical treatment may range from simple aspiration to complete excision of the lymph node
  • However, simple aspiration using FNA can result in the formation of a fistula and hence should be avoided
  • Also, a complete excision can cause facial nerve injury, in case of large matted lymph nodes. Facial nerve is present in the neck region and is prone to injury during a neck surgery

How can Scrofula be Prevented?

In order to ensure the prevention of Scrofula, a proper treatment of tuberculosis (TB) must be undertaken, in order to prevent or control the infection spread.

  • All patients with latent TB infection should be treated with a course of isoniazid for 9 months
  • The best strategy to prevent an infection of tuberculosis bacteria is to avoid contact with infected individuals, especially in closed and crowded areas
  • If such a contact is unavoidable, then proper respiratory protective equipment, such as a mask, should be used
  • Presently, there are tuberculosis vaccines available, called bacilli Calmette-Guerin (BCG). However, the effectiveness of the vaccine is currently being debated upon; even though, it has been shown to reduce the rate of TB in children living in developing countries
  • It is essential to treat individuals, who have come into active contact with TB-infected individuals (those having confirmed tuberculosis infection)

What is the Prognosis of Scrofula? (Outcomes/Resolutions)

The prognosis of Scrofula is dependent upon the type of bacteria causing the infection, and how well the infection responds to treatment measures. In general, with appropriate medical and surgical treatment, the prognosis is good.

Additional and Relevant Useful Information for Scrofula:

  • Tuberculosis (TB) is caused by the infection of Mycobacterium tuberculosis bacteria. It is a highly contagious infection that spreads through the air, when an infected individual sneezes, coughs, sings, or even speaks

The following article link will help you understand tuberculosis infection:

https://www.dovemed.com/diseases-conditions/tuberculosis-tb/

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Krish Tangella MD, MBA picture
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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team

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