| Mycobacterial cervical lymphadenitis | |
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| Other names | Scrofula, scrophula, struma, the king's evil |
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| Scrofula of the neck, showing characteristic bumps on the skin | |
| Specialty | Infectious disease |
The disease mycobacterial cervical lymphadenitis, also known as scrofula and historically as king's evil, involves a lymphadenitis of the cervical lymph nodes associated with tuberculosis as well as nontuberculous (atypical) mycobacteria.
Scrofula is the term used for lymphadenopathy of the neck, usually as a result of an infection in the lymph nodes known as lymphadenitis. It can be caused by tuberculous or nontuberculous mycobacteria. About 95% of the scrofula cases in adults are caused by Mycobacterium tuberculosis , most often in immunocompromised patients (about 50% of cervical tuberculous lymphadenopathy). In immunocompetent children, scrofula is often caused by atypical mycobacteria ( Mycobacterium scrofulaceum) and other nontuberculous mycobacteria (NTM). Unlike the adult cases, only 8% of cases in children are tuberculous.[ citation needed ] With the stark decrease of tuberculosis in the second half of the 20th century, scrofula became a less common disease in adults, but remained common in children. With the appearance of HIV/AIDS, however, it has shown a resurgence, and can affect patients at all stages of the disease. [1] [2]
The most usual signs and symptoms are the appearance of a chronic, painless mass in the neck, which is persistent and usually grows with time. The mass is referred to as a "cold abscess", because there is no accompanying local color or warmth and the overlying skin acquires a violaceous (bluish-purple) color. NTM infections do not show other notable constitutional symptoms, but scrofula caused by tuberculosis is usually accompanied by other symptoms of the disease, such as fever, chills, malaise and weight loss in about 43% of the patients. As the lesion progresses, skin becomes adhered to the mass and may rupture, forming a sinus and an open wound. The fatal outcome some patients experienced in earlier times was due to a cheese-like presentation of the lungs and the King's Evil lesions. It was also associated with pulmonary tuberculosis. [3]
Cervical lymphadenitis is commonly caused by an infection of mycobacteria in the head region. This disease is very inconsistent; cases can have different laboratory findings. Sometimes the disease can occur due to tuberculosis disease. However it is vital that, on a case-by-case basis, it is determined whether the cause is tuberculous or nontuberculous mycobacteria, as treatment often differs between the two forms. [4]
Diagnosis is usually performed by needle aspiration biopsy or excisional biopsy of the mass and the histological demonstration of stainable acid-fast bacteria in the case of infection by M. tuberculosis (Ziehl–Neelsen stain), or the culture of NTM using specific growth and staining techniques.
The classical histologic pattern of scrofula features caseating granulomas with central acellular necrosis (caseous necrosis) surrounded by granulomatous inflammation with multinucleated giant cells. Although tuberculous and non tuberculosis lymphadenitis are morphologically identical, the pattern is somewhat distinct from other causes of bacterial lymphadenitis. [5]
King's Evil was known as a frequent disorder in the 17th century, and was believed to be caused by bad blood coagulating in spongy organs such as the thyroid and the lymph nodes. Physicians also believed the disease could be inherited and could result from parents' untreated venereal infections. [6]
The treatment for mycobacterial cervical lymphadenitis consisted primarily of small incisions to remove the surrounding soft tissue and/or the abnormal mass. Until the 18th century, many doctors thought the only way to cure the disease was to be touched by a member of a royal family. In both France and England, the kings who were thought to have an inherited miraculous power to cure the illness, touched crowds of infected people. The 'touchings' began in France during the reign of King Philip I (1060–1108) and in England during the reign of King Henry I (1100–1135). This act of public healing by powerful kings and royal family members encouraged the nickname "King's Evil". [7] After the touching, the sovereign presented the affected person with an angel on a gold-plated coin that was to be hung around the infected person by a ribbon. This was used as a way of warding off the disease. This coin could have weighed as much as 5 grams and was considered a touch piece of great value. [8] At age three the English lexicographer Samuel Johnson, for example, was treated for scrofula in this way, touched by Queen Anne and presented with a piece of gold, unfortunately, to no effect. [9]
The royal touch and surgical removal were not the only methods of healing employed: Scrophularia nodosa (common name: Figwort), which has nodular roots that resemble the swollen lymph nodes of the affected, was thought to be useful in treating the disease, according to the doctrine of signatures – the plant being hung around the neck of the affected. Figwort does, in fact, contain compounds that can help decrease inflammation, irritation and discomfort. [10]
Treatments are highly dependent on the kind of infection. Surgical excision of the scrofula does not work well for M. tuberculosis infections, and has a high rate of recurrence and formation of fistulae.[ citation needed ] Furthermore, surgery may spread the disease to other organs. The best approach is to use conventional treatment of tuberculosis with antibiotics. The cocktail-drug treatment of tuberculosis (and inactive meningitis) includes rifampicin along with pyrazinamide, isoniazid, ethambutol, and streptomycin ("PIERS"). Scrofula caused by NTM, however, responds well to surgery, but is usually resistant to antibiotics.[ citation needed ] The affected nodes can be removed either by repeated aspiration, curettage or total excision (with the risk in the latter procedure, however, often causing unsightly scarring, damage to the facial nerve, or both).[ citation needed ]
Many different therapeutic options exist, particularly regarding non tuberculosis mycobacterial infections, such as incision and drainage, aspiration biopsy and chemotherapy. All of these methods have proved to result in a cure of the disease. However different treatments can cause different side effects along the way to recovery. Some of these side effects include facial nerve injury and scarring. Therefore, the course of treatment is tailored to each patient, taking into account their history as well as the severity of infection. [11]
With adequate treatment, clinical remission is practically 100%. In NTM infections, with adequate surgical treatment, clinical remission is greater than 95%. It is recommended that persons in close contact with the diseased person, such as family members, be tested for tuberculosis.
The term 'cervical' refers to the cervical lymph nodes in the neck; it is unrelated to the cervix. The alternative name scrofula comes from the medieval Latin scrōfula, diminutive of scrōfa, meaning brood sow , because swine were supposed to be subject to the complaint.[ citation needed ], or because the line of elevated lymph nodes was thought to resemble the belly of a breastfeeding sow.
In the beginning of the Modern Age some Western Europeans[ citation needed ] believed that royal touch, the touch of the sovereign of England or France, could cure diseases owing to the divine right of sovereigns. Henry VI of England is alleged to have cured a girl with it. Scrofula was therefore also known as the King's evil. From 1633, the Book of Common Prayer of the Anglican Church contained a ceremony for this, and it was traditional for the monarch (king or queen) to present to the touched person a coin—usually an angel, a gold coin the value of which varied from about 6 shillings to about 10 shillings. In England this practice continued until the early 18th century, and was continued by the Jacobite pretenders until the extinction of the House of Stuart with the death of the pretender Henry IX. King Henry IV of France is reported as often touching and healing as many as 1,500 individuals at a time. Queen Anne touched the infant Samuel Johnson in 1712, [12] but King George I put an end to the practice as being "too Catholic".[ citation needed ] The kings of France continued the custom until Louis XV stopped it in the 18th century, though it was briefly revived by Charles X in 1825.
Physicians, healers, and patent medicine sellers offered a wide range of cures for scrofula or the King's Evil. Since ancient times, the highly toxic heavy metal mercury, referred to as cinnabar, quicksilver or calomel, was administered as an ointment or pill or inhaled as a vapor to treat skin diseases. Mercury taken internally induced vomiting and sweating, reactions believed to cure the disease. In 1830, the New-York Medical and Physical Journal continued to recommend mercury as the best cure for scrofula, stating it caused an irritation that would counteract the disease and increased the working of the glands. [13] Alternative treatments were also offered. Many rejected the harsh side effects of mercury, claiming their cures were made of "natural" or "vegetable" ingredients. Patent medicines labeled as sarsaparilla were recommended for scrofula. [14]
Examples of treatments recommended between the 17th and 19th century include the following:
In 1924, French historian Marc Bloch wrote a book on the history of the royal touch: The Royal Touch: Sacred Monarchy and Scrofula in England and France (original in French). [19]
A three-year-old healthy young female presented with a bilateral cervical lymph node enlarged. The patient was admitted to the hospital after tuberculosis skin test became positive and further examination showed several other enlarged lymph nodes near her neck. At the hospital, she underwent an exploration surgery where they excised part of her presented lymph node and drained her retropharynx. The drained retropharynx grew methicillin-resistant Staphylococcus epidermidis and Streptococcus mitis. After these findings, the patient received oral linezolid for ten days and had antimicrobial drug therapy for 14 days. Once the patient returned for a follow-up appointment, the lymph node had only slightly decreased in size. Due to this, it had to be completely removed from her neck. Bacterial cultivation of tissue from the excised lymph node resulted in the growth of "atypical Mycobacteria", which were identified by 16S gene sequencing as Mycobacterium florentinum . After she recovered and went home, there were no repeat signs that the infection was back for over a year. [20]
Tuberculosis (TB), also known colloquially as the "white death", or historically as consumption, is an infectious disease usually caused by Mycobacterium tuberculosis (MTB) bacteria. Tuberculosis generally affects the lungs, but it can also affect other parts of the body. Most infections show no symptoms, in which case it is known as latent tuberculosis. Around 10% of latent infections progress to active disease which, if left untreated, kill about half of those affected. Typical symptoms of active TB are chronic cough with blood-containing mucus, fever, night sweats, and weight loss. Infection of other organs can cause a wide range of symptoms.
Kikuchi disease was described in 1972 in Japan. It is also known as histiocytic necrotizing lymphadenitis, Kikuchi necrotizing lymphadenitis, phagocytic necrotizing lymphadenitis, subacute necrotizing lymphadenitis, and necrotizing lymphadenitis. Kikuchi disease occurs sporadically in people with no family history of the condition.
Mycobacterium is a genus of over 190 species in the phylum Actinomycetota, assigned its own family, Mycobacteriaceae. This genus includes pathogens known to cause serious diseases in mammals, including tuberculosis and leprosy in humans. The Greek prefix myco- means 'fungus', alluding to this genus' mold-like colony surfaces. Since this genus has cell walls with a waxy lipid-rich outer layer that contains high concentrations of mycolic acid, acid-fast staining is used to emphasize their resistance to acids, compared to other cell types.
Adenitis is a general term for an inflammation of a gland. Often it is used to refer to lymphadenitis which is the inflammation of a lymph node.
Nontuberculous mycobacteria (NTM), also known as environmental mycobacteria, atypical mycobacteria and mycobacteria other than tuberculosis (MOTT), are mycobacteria which do not cause tuberculosis or leprosy/Hansen's disease. NTM are able to cause pulmonary diseases that resemble tuberculosis. Mycobacteriosis is any of these illnesses, usually meant to exclude tuberculosis. They occur in many animals, including humans and are commonly found in soil and water.
Lymphadenopathy or adenopathy is a disease of the lymph nodes, in which they are abnormal in size or consistency. Lymphadenopathy of an inflammatory type is lymphadenitis, producing swollen or enlarged lymph nodes. In clinical practice, the distinction between lymphadenopathy and lymphadenitis is rarely made and the words are usually treated as synonymous. Inflammation of the lymphatic vessels is known as lymphangitis. Infectious lymphadenitis affecting lymph nodes in the neck is often called scrofula.
Peripheral tuberculous lymphadenitis is a form of tuberculosis infection occurring outside of the lungs. In general, it describes tuberculosis infection of the lymph nodes, leading to lymphadenopathy. When cervical lymph nodes are affected, it is commonly referred to as "Scrofula." A majority of tuberculosis infections affect the lungs, and extra-pulmonary tuberculosis infections account for the remainder; these most commonly involve the lymphatic system. Although the cervical region is most commonly affected, tuberculous lymphadenitis can occur all around the body, including the axillary and inguinal regions.
Lymph node biopsy is a test in which a lymph node or a piece of a lymph node is removed for examination under a microscope.
The royal touch was a form of laying on of hands, whereby French and English monarchs touched their subjects, regardless of social classes, with the intent to cure them of various diseases and conditions. The thaumaturgic touch was most commonly applied to people suffering from tuberculous cervical lymphadenitis, and exclusively to them from the 16th century onwards. The disease rarely resulted in death and often went into remission on its own, giving the impression that the monarch's touch cured it. The claimed power was most notably exercised by monarchs who sought to demonstrate the legitimacy of their reign and of their newly founded dynasties.
Mycobacterium avium-intracellulare infection (MAI) is an atypical mycobacterial infection, i.e. one with nontuberculous mycobacteria or NTM, caused by Mycobacterium avium complex (MAC), which is made of two Mycobacterium species, M. avium and M. intracellulare. This infection causes respiratory illness in birds, pigs, and humans, especially in immunocompromised people. In the later stages of AIDS, it can be very severe. It usually first presents as a persistent cough. It is typically treated with a series of three antibiotics for a period of at least six months.
Mycobacteroides abscessus is a species of rapidly growing, multidrug-resistant, nontuberculous mycobacteria (NTM) that is a common soil and water contaminant. Although M. abscessus most commonly causes chronic lung infection and skin and soft tissue infection (SSTI), it can also cause infection in almost all human organs, mostly in patients with suppressed immune systems. Amongst NTM species responsible for disease, infection caused by M. abscessus complex are more difficult to treat due to antimicrobial drug resistance.
Mycobacterium bohemicum is a species of the phylum Actinomycetota, belonging to the genus Mycobacterium.
Mycobacterium fortuitum is a nontuberculous species of the phylum Actinomycetota, belonging to the genus Mycobacterium.
Mycobacterium avium complex is a group of mycobacteria comprising Mycobacterium intracellulare and Mycobacterium avium that are commonly grouped because they infect humans together; this group, in turn, is part of the group of nontuberculous mycobacteria. These bacteria cause Mycobacterium avium-intracellulare infections or Mycobacterium avium complex infections in humans. These bacteria are common and are found in fresh and salt water, in household dust and in soil. MAC bacteria usually cause infection in those who are immunocompromised or those with severe lung disease.
Mycobacterium kansasii is a bacterium in the Mycobacterium genus. It is an environmental bacteria that causes opportunistic infections in humans, and is one of the leading mycobacterial causes of human disease after tuberculosis and leprosy.
The history of tuberculosis encompasses the origins of the disease, tuberculosis (TB) through to the vaccines and treatments methods developed to contain and mitigate its impact.
Mycobacterium scrofulaceum is a species of Mycobacterium.
Cervical lymphadenopathy refers to lymphadenopathy of the cervical lymph nodes. The term lymphadenopathy strictly speaking refers to disease of the lymph nodes, though it is often used to describe the enlargement of the lymph nodes. Similarly, the term lymphadenitis refers to inflammation of a lymph node, but often it is used as a synonym of lymphadenopathy.
Extrapulmonary tuberculosis is tuberculosis (TB) within a location in the body other than the lungs. It accounts for an increasing fraction of active cases, from 20 to 40% according to published reports, and causes other kinds of TB. These are collectively denoted as "extrapulmonary tuberculosis". Extrapulmonary TB occurs more commonly in immunosuppressed persons and young children. In those with HIV, this occurs in more than 50% of cases. Notable extrapulmonary infection sites include the pleura, the central nervous system, the lymphatic system, the genitourinary system, and the bones and joints, among others.
A lung cavity or pulmonary cavity is an abnormal, thick-walled, air-filled space within the lung. Cavities in the lung can be caused by infections, cancer, autoimmune conditions, trauma, congenital defects, or pulmonary embolism. The most common cause of a single lung cavity is lung cancer. Bacterial, mycobacterial, and fungal infections are common causes of lung cavities. Globally, tuberculosis is likely the most common infectious cause of lung cavities. Less commonly, parasitic infections can cause cavities. Viral infections almost never cause cavities. The terms cavity and cyst are frequently used interchangeably; however, a cavity is thick walled, while a cyst is thin walled. The distinction is important because cystic lesions are unlikely to be cancer, while cavitary lesions are often caused by cancer.
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