Scrofula



Scrofula (Scrophula or Struma) refers to a variety of skin diseases; in particular, a form of tuberculosis, affecting the lymph nodes of the neck. In adults it is caused by Mycobacterium tuberculosis and in children by nontuberculous mycobacteria. The word comes from the Latin scrofulae, meaning brood sow.

History
In the Middle Ages it was believed that "royal touch", the touch of the sovereign of England or France, could cure the disease. Scrofula was therefore also known as the King's Evil. The kings were thought to have received this power from descent from Edward the Confessor, who, according to some legends, received it from Saint Remigius. 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. 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 Dr. Samuel Johnson in 1712, but King George I put an end to the practice as being "too Catholic." The kings of France continued the custom until Louis XV stopped in the 1700s, though it was briefly revived to universal derision in 1825.

In 1768 the Englishman John Morley produced a handbook "Essay on the nature and cure of scrophulous disorders, commonly called the King's Evil". The book starts by listing the typical symptoms and indications of how far the disease had progressed. It then goes into detail with a number of case studies, describing the specific case of the patient, the various treatments used and their effectiveness. The forty-second edition was printed in 1824.

In 1924, the 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).

The disease
Scrofula is the term used for tuberculosis of the neck, or, more precisely, a cervical tuberculous lymphadenopathy. Scrofula is usually a result of an infection in the lymph nodes, known as lymphadenitis and is most often observed in immunocompromised patients (about 50% of cervical tuberculous lymphadenopathy). About 95% of the scrofula cases in adults are caused by Mycobacterium tuberculosis, but only 8% in children. The rest are caused by atypical mycobacterium or nontuberculous mycobacterium (NTM). With the stark decrease of tuberculosis in the second half of the 20th century, scrofula became a very rare disease. With the appearance of AIDS, however, it has shown a resurgence, and presently affects about 5% of severely immunocompromised patients.

Signs and symptoms
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.

Diagnosis
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.

Therapy
Treatment approaches 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. Furthermore, surgery may spread the disease to other organs. The best approach then is to use conventional treatment of tuberculosis with antibiotics. Scrofula caused by NTM, on the other hand, responds well to surgery, but is usually resistant to antibiotics. The affected nodes can be removed either by repeated aspiration, curettage or total excision (with the risk in the latter procedure, however, of causing cosmetically negative effects or damage to the facial nerve, or both).

Prognosis
Prognosis: 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, should undergo testing for tuberculosis.