Sunday, April 14, 2013

Buruli Ulcer - Part I (Overview)


What is Buruli Ulcer: Buruli ulcer is a devastating bacterial infection caused by Mycobacterium ulcerans.  It generally affects the skin and soft tissues but can sometimes affect the bone. The legs and arms are the most common place for infection. Mycobacterium ulcerans produces a toxin – mycolactone – that kills the cells surrounding the site of infection and results in extensive destruction of  the skin and soft tissue.  It also interferes with the immune response. Mycobacterium ulcerans belongs to the same family of bacteria that causes tuberculosis and leprosy.

Other names for Buruli Ulcer: • Bairnsdale ulcer • Daintree ulcer • Mossman ulcer • Searl ulcer
How do you get it Although the exact route of transmission is not yet known, a common theory is that Mycobacterium ulcerans enters a person (or host) through a break in the skin that may have occurred by an insect bite, cut, or wound.   Living organisms such as aquatic insects, adult mosquitoes or other biting arthropods may also have a role in transmission, such as serving as carrier or host, although this has not yet been proven.

Who is at risk All races appear to be affected equally.
There is no difference in the rates of infection among males and females. Children between the ages 5 - 15 years appear to be most at risk, however, any age group may be affected.
Persons who have direct daily exposure to slow-flowing or stagnant water appear to be at greater risk for infection. Although still being studied, co-infection with HIV and M. ulcerans (both of which suppress the immune system) may speed up the progression of Buruli ulcer as well as negatively impact response to antibiotic treatment.   

What are the symptoms Symptoms typically appear 5-12 weeks after exposure but may take up to 6 months to develop. Buruli ulcer usually begins as a single, painless, occasionally pruritic, dermal papule or subcutaneous nodule.  This is known as the pre-ulcerative stage.   The edematous variant of the disease is characterized by rapid onset of extensive swelling that may involve an entire arm or leg which then results in very large ulcer. Fever or swollen lymph nodes (lymphadenopathy) are rare as the immunosuppressive properties of the mycolactone toxin allow the disease to progress without pain or other symptoms.  Ulcerations are generally painless unless complicated by secondary infection. Over a period of weeks to months, the skin breaks down to form a necrotic ulcer with extensively undermined edges.  This is the ulcerative stage. Approximately 90% of lesions occur on the arms and legs, with 60% occurring on the legs.  The other 10% can occur on the chest, back and face. Buruli ulcers destroy nerves, appendages (ie. fingers, toes), blood vessels and may invade bone. Lesions eventually heal but leave significant scarring that usually results in permanent disfigurement and/or disability as well as the risk of life-threatening secondary infections. Cancerous lesions may occur in skin, soft tissue, or bone due to spread through the vasculature or lymphatics.
Clinical symptoms of Buruli ulcer disease. (a) nodule on abdomen of a young child (b) non-ulcerative edematous swelling of child's hand (c) ulcer covering most of a young child's arm (d) early stage plaque

Long term effects from the disease Skin and soft tissue necrosis (death of the skin and surrounding cells) can be extensive, involving as much as 15% of the infected person's skin surface.


Although deaths associated with Buruli Ulcer are low, the risk of acquiring the disease is high and therefore the risk of permanent disfigurement and disability is significant.  This can then have considerable emotional and socioeconomic impact on those affected as well as their family and community members. 

Treatment involves long hospital stays, antibiotic regimens, and surgical procedures, which are resources often limited in endemic areas. 

Early diagnosis and treatment is critical as up to 80% of cases found early can be cured with a combination of antibiotics.


References:

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Medical science has proven time and again that when the resources are provided, great progress in the treatment, cure, and prevention of disease can occur.

- Michael J. Fox


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