What are the other Names for this Condition? (Also known as/Synonyms)

• MCL (Mucocutaneous Leishmaniasis)

What is Mucocutaneous Leishmaniasis? (Definition/Background Information)

• Leishmaniasis is a widespread, global disease that is caused by protozoa (of the Leishmania genus), which is transmitted through the bite of an infected female sandfly (a type of insect) in the tropical and subtropical regions
• There are 3 main types of Leishmaniasis that include:
  • Cutaneous Leishmaniasis, which affects the skin
  • Mucocutaneous Leishmaniasis, which affects the skin and mucous membrane
  • Visceral Leishmaniasis, which affects the entire body (systemic)
• Mucocutaneous Leishmaniasis (MCL) is the most uncommon form of Leishmaniasis. It is similar to the cutaneous form, but then the infection invades (after many years) the mucus membranes causing severe destruction of skin and tissues of the mouth and nasal cavity
• It is caused by nearly 4 different species of Leishmania protozoa, which are mainly found in certain South American nations. Mucocutaneous Leishmaniasis is a poorly understood infection, but is believed to develop due to an inadequate or complete lack of treatment of Cutaneous Leishmaniasis
• Mucocutaneous Leishmaniasis is characterized by skin and mucosal involvement with formation of lesions that spread locally and severely mutilate the face. This may be accompanied by fever, fatigue, and general illness. The complications may include secondary infections and complete destruction of the face (particularly the region around the mouth and nose)
• The treatment of Mucocutaneous Leishmaniasis involves medications and topical applications. It is based on the type and severity of the infection. Since, human infection can be caused by many different species of Leishmania; the treatment also depends upon the geographical location. Restorative surgery may be required for severe cases
• The prognosis depends upon the promptness in providing appropriate treatment. The prognosis of Mucocutaneous Leishmaniasis is generally satisfactory, but severe complications can result in irreversible facial damage and disfigurement

Who gets Mucocutaneous Leishmaniasis? (Age and Sex Distribution)

Individuals of all ages are at risk for Mucocutaneous Leishmaniasis infection, if they are exposed to sandfly bites. There is no gender, race, or ethnicity preference noted. However, Mucocutaneous Leishmaniasis is endemic to certain regions of the world as indicated below:

• Latin American countries consisting of Brazil, Bolivia, and Peru account for nearly 90% of the cases; other nations include Colombia, Paraguay, and Venezuela
• African countries including Ethiopia, Sudan, Kenya, and Namibia

What are the Risk Factors for Mucocutaneous Leishmaniasis? (Predisposing Factors)

Presently, the major risk factor for Mucocutaneous Leishmaniasis is an inadequate or improper treatment of Cutaneous Leishmaniasis. However, in some cases, the involvement of skin and mucosal membranes take place simultaneously; the cutaneous form arises with mucocutaneous form.

In general, the risk factors for Mucocutaneous Leishmaniasis may include:

• Living in or visiting certain south American and African countries
• Climatic conditions and environmental changes can increase the risk of Leishmaniasis. These include deforestation, development and expansion of cities, and construction of dams
• According to the World Health Organization (WHO), certain socio-economic indicators point to a higher risk of infection. These include:
  • Poverty
  • Malnutrition
  • Famine
  • Illiteracy
  • Lack of sanitation and proper hygiene
  • Large-scale migration and settlements
• Individuals with weak immune system owing to factors, such as HIV infection or other diseases, are more likely to contract Leishmaniasis
• Leishmaniasis is more common in the villages and small towns (especially in the outskirts) than in the cities

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Mucocutaneous Leishmaniasis? (Etiology)

Leishmaniasis is an infectious disease caused by the bite of sandflies; small flying insects that are smaller than mosquitoes and less noisy. The sandflies transmit Leishmania organisms, which are protozoan parasites, in blood causing the infection.

• However, the exact cause of development of Mucocutaneous Leishmaniasis, which occurs sometimes after a few months or after several years (even over 20 years) due to the poor treatment or an absence of treatment of Cutaneous Leishmaniasis, is generally unknown. It is believed that the parasite invades the mucus membranes after first attacking the skin
• Nevertheless, not all cases of Mucocutaneous Leishmaniasis arise from the cutaneous form. In some cases, mucocutaneous and cutaneous types occur together; while, in others the mucocutaneous type may immediately follow the cutaneous type
• More than 4 different species of Leishmania parasites have been identified and studied. Some of these include:
  • L. amazonensis
  • L. (Viannia) braziliensis
  • L. (V.) guyanensis
  • L. (V.) panamensis

What are the Signs and Symptoms of Mucocutaneous Leishmaniasis?

The signs and symptoms of Mucocutaneous Leishmaniasis include:

• Initial symptoms may resemble those of Cutaneous Leishmaniasis
• Affects the skin and mucus membranes, mainly of the oral cavity (mouth and throat) and nose
• Skin lesions (due to Cutaneous Leishmaniasis) may heal spontaneously, but reappear at other locations as mucocutaneous lesions that can be severely damaging to the mucus membranes and skin
• Stuffy nose and breathing difficulties that are persistent
• Bleeding from the nose
• Formation of multiple ulcers that spread across the mouth and nose; mucosal perforations
• Bleeding and ulceration; there is no involvement of the bone, but skin and tissue involvement is present
• Disfigurement of the face and mouth

How is Mucocutaneous Leishmaniasis Diagnosed?

A physical examination, assessment of the signs and symptoms, and medical history evaluation of the infected individual may be indicative of Mucocutaneous Leishmaniasis. However, the healthcare provider may perform the following tests to definitely diagnose the condition:

• Certain blood tests to detect antibodies against the parasite
• Montenegro skin test: It is generally not a very effective method to aid in the diagnosis
• DNA testing (hybridization) and PCR
• Biopsy of skin lesion
• Detection of pathogen using staining methods
• Examining tissues under a microscope to detect the parasite

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Mucocutaneous Leishmaniasis?

Typically, Mucocutaneous Leishmaniasis is not a fatal condition. But, if it is left untreated (or inadequately treated), then it may lead to complications such as:

• Severe scarring of skin, distortion of the facial profile (may be severe and disabling)
• Facial disfigurement may necessitate cosmetic surgery
• Secondary infections of bacteria, virus, or fungus
• Severe bleeding
• Deadly infections due to immune system damage
• Social ostracisation and humiliation due to severe disfigurement, especially of the face

How is Mucocutaneous Leishmaniasis Treated?

The treatment for Mucocutaneous Leishmaniasis differs from one individual to another. Proper consultation with the infectious disease consultants and public health officers are generally recommended before treatment starts. The treatment measures include:

• Oral medicines and topical ointments/applications for skin lesions: The ulcers do not go away on their own without suitable treatment
• If the ulcers heal and cause cosmetic disfigurement, then plastic surgery may be performed. A restorative surgery may be required for the face and nose
• Intravenous medications (that may include intramuscular injections)
• For fungal (secondary) infections that develop due to Leishmaniasis, antifungal medication are prescribed

The medications administered vary depending upon the type of Leishmania species causing the infection.

How can Mucocutaneous Leishmaniasis be Prevented?

Currently, no vaccination has been discovered to prevent Leishmaniasis. Research is being undertaken to develop a vaccine at the earliest. Nevertheless, the following preventive and precautionary measures may be taken to reduce the spread and impact of Mucocutaneous Leishmaniasis:

• Undertake immediate, effective, and proper treatment of Cutaneous Leishmaniasis. This is a very important steps towards preventing Mucocutaneous Leishmaniasis
• Avoid or limit exposure to sandfly bites
• Wear protective clothing, such as long sleeves, long pants, and socks, to protect the skin from the insect bites; minimize exposed skin areas
• Use insect repellents containing DEET, permethrin, or deltamethrin; one’s clothing and bed can be suitably sprayed with the repellents
• Prevent entry of sandflies into homes by keeping the windows and doors suitably closed; use finely-meshed bed nets (that are tucked into the mattress) while sleeping
• Sandflies are more active during dusk or at night; knowing their pattern, one can take steps to minimize outdoor activities or take suitable preventive measures against the bites accordingly
• Dogs can be fitted with collars containing insect repellents
• Local strategies (specific to each community or locality) and community development to eliminate Leishmaniasis should be encouraged

What is the Prognosis of Mucocutaneous Leishmaniasis? (Outcomes/Resolutions)

• An early diagnosis and prompt treatment of Mucocutaneous Leishmaniasis generally results in a satisfactory outcome
• A delayed treatment or incomplete treatment may lead to permanently disfigured or severely mutilated face
• In general, the prognosis of Mucocutaneous Leishmaniasis is better than the visceral form, but worse than the cutaneous form of Leishmaniasis

Additional and Relevant Useful Information for Mucocutaneous Leishmaniasis:

Animals, such as dogs, rats, and cattle, can also be infected by Leishmaniasis. According to the World Health Organization, over 70 species of animals act as hosts for the Leishmania organism.