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Saturday, October 19, 2013

Scrofula, the king's evil


Have you ever heard the term "king's evil" and wondered what it means?

Scrofula is the term used for lymphadenopathy of the neck, usually as a result of an infection in the lymph nodes, known as lymphadenitis. About 95% of the scrofula cases in adults are caused by Mycobacterium tuberculosis, most often in immunocompromised patients. In children with normal immune systems, scrofula is often caused by atypical mycobacteria (Mycobacterium scrofulaceum) and other nontuberculous mycobacteria. Unlike the adult cases, only 8% of cases in children are tuberculous.

The most usual symptom of scrofula is a chronic, painless mass in the neck, which 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 bluish-purple color. Scrofula caused by tuberculosis is often accompanied by other symptoms of the disease, such as fever, chills, malaise and weight loss. As the lesion progresses, skin becomes adhered to the mass and may rupture, forming a sinus (a cavity resulting from the destruction of tissue) and an open wound.

Extrapulmonary tuberculosis (tuberculosis occurring somewhere other than the lungs) most often presents as scrofula. Concomitant pulmonary tuberculosis occurs in fewer than 50% of cases.

Treatment depends on the kind of infection. Surgical excision of the scrofula does not work well for M. tuberculosis infections, since recurrences are common; furthermore, surgery may spread the disease to other organs. The best approach is to use conventional treatment of tuberculosis with antibiotics. Scrofula caused by nontuberculous mycobacteria, on the other hand, responds well to surgery (removal of the affected nodes, e.g. by aspiration or by excision), but is usually resistant to antibiotics. 

In the Middle Ages it was believed that royal touch, the touch of the sovereign of England or France, could cure diseases - and especially scrofula. Scrofula was therefore also known as the "King's Evil". William the Lion, King of Scotland is recorded in 1206 as curing a case of scrofula by his touching and blessing a child who had the ailment. Charles I touched around 100 people shortly after his coronation at Holyrood in 1630. Many miraculous cures were recorded, and failures were put down to the sufferer's lack of faith. The divine power of kings was believed to be descended 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 description of this ceremony. 

It was traditional for the monarch (king or queen) to present to the touched person a coin, usually a gold one, which was seen as a sort of amulet, infused with the monarch's divine healing power. Such a touch-piece had to be worn around the neck on a ribbon, otherwise the condition would return. In England this practice of ceremonial touching by monarchs continued until the early 18th century. King Henry IV of France is reported as often touching and (reportedly) healing as many as 1,500 individuals at a time.



 King Henry IV of France. Image from: Wikipedia


Despite widespread vaccinations against tuberculosis, scrofula still occurs today, especially in immunocompromised patients (e.g. AIDS patients) who are susceptible to tuberculosis infections. Occasionally it can occur in immunocompetent persons too, as a recent case from France shows. A previously well 77-year-old woman was admitted to hospital in Toulouse with a six-month history of weight loss, fatigue and low-grade fever, and a painless ulcerated mass on her neck. Cultures of the fluid from the ulcer and the patient’s sputum grew Mycobacterium tuberculosis sensitive to first-line antituberculous drugs. She was treated with antituberculosis drugs for a total of nine months and a year later, she was symptom-free.




(A) Ulcerated mass on the neck of a 77-year-old woman. (B) A computed tomography scan shows necrotic inflammation of the patient’s cervical lymph nodes (asterisks) and a thrombosis (blood clot) of her left internal jugular vein (arrowhead). Image from: Moulis & Martin-Blondel 2012



Literature: 

Moulis G and Martin-Blondel G (2012) Scrofula, the king's evil. CMAJ. 184(9): 1061. doi: 10.1503/cmaj.111519 

Wikipedia: Tuberculous cervical lymphadenitis


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