Scrofula
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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.
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History
This disease is also called "The King's Evil," as it was believed in the Middle Ages that the touch of the sovereign of England or France could cure it, they having received this power from descent from Edward the Confessor, who, according to some legends, received it from Saint Remigius. The original 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 1825.
The disease
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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 (ca. 50% of cervical tuberculous lymphadenopaty). In ca. 95% of the scrofula cases in adults are caused by Mycobacterium tuberculosis and 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 resurged, and presently affects ca. 5% of severely immunocompromised patients.
Signs and symptoms
Most usual signs and symptoms are the appearance of a chronic, painless mass in the neck, which is persisting and usually grows with time. The mass is referred as a cold abscess, because there is no accompanying local calor or warmth and the overlying skin acquires a violaceous 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 ca. 43% of the patients. As the lesion progresses, skin becames 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 doesn’t 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 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 later, however, of causing cosmetic effects and/or damage to the facial nerve).
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 close contacts of the diseased persong, such as family members, should undergo testing for tuberculosis.
See also
External links
- Scrofula (http://www.emedicine.com/ent/topic524.htm) from eMedicine medical articlefr:Écrouelles