Paracoccidioidomycosis
Encyclopedia
Paracoccidioidomycosis is a mycosis
caused by the fungus
Paracoccidioides brasiliensis
. Sometimes called South American blastomycosis, paracoccidioidomycosis is caused by a different fungus than that which causes blastomycosis
.
and South America
. The habitat of the infectious agent is not known but appears to be aquatic. In biopsies the fungus appears as a polygemulating yeast with a pilot's wheel-like appearance.
s, lymph node
s, bone and lungs. Unlike other systemic mycoses, it can cause disease in immunocompetent hosts, although immunosuppression increases the aggressiveness of the fungus. Also uniquely, it rarely causes disease in fertile-age women, probably due to a protective effect of estradiol.
Primary infection is thought to be autolimited and almost asymptomatic as histoplasmosis
or Valley Fever. In young people, there is a progressive form of the disease (akin of tuberculous septicemia in tuberculous priminfection) with high prostrating fever, generalized lymphadenopathy
and pulmonary involvement with milliary lesions. This juvenile form has a more severe prognosis even with treatment. The most common form is the so called adult form of paracoccidioidomycosis that is almost certainly a reactivation of the disease.
Painful lesions with a violaceous hue in lips and oral mucosa are common as is cervical lymphadenitis teeming with polygemulating yeasts in the biopsy. In this form, differential diagnosis must be made with mucocutaneous leishmaniasis
, yaws
and TB
.
Pulmonary involvement is also common, it starts as lobar pneumonia
or pleurisy
but without remission at ninth day; the patient remains febrile, cough
s, loses weight and the X rays reveal milliary shadows throughout lung fields. Other organs can be involved, like bones, meninges
, arteries and spleen
but this is very rare.
Diagnosis is made with a biopsy of affected tissue, this shows the characteristic helm-shaped yeasts and culture shows the agent. Serology is also used in endemic areas.
are the traditional remedies to paracoccidiodomycosis. They were introduced by Oliveira Ribeiro and used for more than fifty years with good results. The most used sulfa drugs in this infection are sulfadimethoxime, sulfadiazine
and co-trimoxazole
. This treatment is generally safe but several adverse effects can appear, the most severe of which are the Stevens Johnson Syndrome and agranulocytosis
. Similarly to tuberculosis
treatment, it must be continued for up to 3 years to eradicate the fungus, and relapse and treatment failures aren't unusual.
Antifungal drugs like amphotericin B
or ketoconazole
are also effective in clearing the infection but they are very expensive compared with sulfonamides.
During therapy fibrosis
can appear and a surgery be needed to correct this. Other possible complication is Addisonian crisis. The death rate is approximately ten percent.
, Alfonso Splendore, and Floriano Paulo de Almeida, who first characterized the disease in Brazil in the early 20th century.
Mycosis
A mycosis is a fungal infection of animals, including humans. Mycoses are common, and a variety of environmental and physiological conditions can contribute to the development of fungal diseases...
caused by the fungus
Fungus
A fungus is a member of a large group of eukaryotic organisms that includes microorganisms such as yeasts and molds , as well as the more familiar mushrooms. These organisms are classified as a kingdom, Fungi, which is separate from plants, animals, and bacteria...
Paracoccidioides brasiliensis
Paracoccidioides brasiliensis
Paracoccidioides brasiliensis is a dimorphic species of fungus responsible for paracoccidioidomycosis. While the route of infection is assumed to be via inhalation, the environmental habit of P. brasiliensis is unknown...
. Sometimes called South American blastomycosis, paracoccidioidomycosis is caused by a different fungus than that which causes blastomycosis
Blastomycosis
Blastomycosis is a fungal infection caused by the organism Blastomyces dermatitidis...
.
Agent
P. brasiliensis is a thermally-dimorphic fungus distributed in BrazilBrazil
Brazil , officially the Federative Republic of Brazil , is the largest country in South America. It is the world's fifth largest country, both by geographical area and by population with over 192 million people...
and South America
South America
South America is a continent situated in the Western Hemisphere, mostly in the Southern Hemisphere, with a relatively small portion in the Northern Hemisphere. The continent is also considered a subcontinent of the Americas. It is bordered on the west by the Pacific Ocean and on the north and east...
. The habitat of the infectious agent is not known but appears to be aquatic. In biopsies the fungus appears as a polygemulating yeast with a pilot's wheel-like appearance.
Disease
Paracoccidioidomycosis is a systemic mycosis caused by the dimorphic fungus Paracoccidioides. It frequently involves mucous membraneMucous membrane
The mucous membranes are linings of mostly endodermal origin, covered in epithelium, which are involved in absorption and secretion. They line cavities that are exposed to the external environment and internal organs...
s, lymph node
Lymph node
A lymph node is a small ball or an oval-shaped organ of the immune system, distributed widely throughout the body including the armpit and stomach/gut and linked by lymphatic vessels. Lymph nodes are garrisons of B, T, and other immune cells. Lymph nodes are found all through the body, and act as...
s, bone and lungs. Unlike other systemic mycoses, it can cause disease in immunocompetent hosts, although immunosuppression increases the aggressiveness of the fungus. Also uniquely, it rarely causes disease in fertile-age women, probably due to a protective effect of estradiol.
Primary infection is thought to be autolimited and almost asymptomatic as histoplasmosis
Histoplasmosis
Histoplasmosis is a disease caused by the fungus Histoplasma capsulatum. Symptoms of this infection vary greatly, but the disease primarily affects the lungs...
or Valley Fever. In young people, there is a progressive form of the disease (akin of tuberculous septicemia in tuberculous priminfection) with high prostrating fever, generalized lymphadenopathy
Lymphadenopathy
Lymphadenopathy is a term meaning "disease of the lymph nodes." It is, however, almost synonymously used with "swollen/enlarged lymph nodes". It could be due to infection, auto-immune disease, or malignancy....
and pulmonary involvement with milliary lesions. This juvenile form has a more severe prognosis even with treatment. The most common form is the so called adult form of paracoccidioidomycosis that is almost certainly a reactivation of the disease.
Painful lesions with a violaceous hue in lips and oral mucosa are common as is cervical lymphadenitis teeming with polygemulating yeasts in the biopsy. In this form, differential diagnosis must be made with mucocutaneous leishmaniasis
Leishmaniasis
Leishmaniasis is a disease caused by protozoan parasites that belong to the genus Leishmania and is transmitted by the bite of certain species of sand fly...
, yaws
Yaws
Yaws is a tropical infection of the skin, bones and joints caused by the spirochete bacterium Treponema pallidum pertenue...
and TB
Tuberculosis
Tuberculosis, MTB, or TB is a common, and in many cases lethal, infectious disease caused by various strains of mycobacteria, usually Mycobacterium tuberculosis. Tuberculosis usually attacks the lungs but can also affect other parts of the body...
.
Pulmonary involvement is also common, it starts as lobar pneumonia
Lobar pneumonia
Lobar pneumonia is a form of pneumonia that affects a large and continuous area of the lobe of a lung.It is one of the two anatomic classifications of pneumonia .- Symptoms :...
or pleurisy
Pleurisy
Pleurisy is an inflammation of the pleura, the lining of the pleural cavity surrounding the lungs. Among other things, infections are the most common cause of pleurisy....
but without remission at ninth day; the patient remains febrile, cough
Cough
A cough is a sudden and often repetitively occurring reflex which helps to clear the large breathing passages from secretions, irritants, foreign particles and microbes...
s, loses weight and the X rays reveal milliary shadows throughout lung fields. Other organs can be involved, like bones, meninges
Meninges
The meninges is the system of membranes which envelopes the central nervous system. The meninges consist of three layers: the dura mater, the arachnoid mater, and the pia mater. The primary function of the meninges and of the cerebrospinal fluid is to protect the central nervous system.-Dura...
, arteries and spleen
Spleen
The spleen is an organ found in virtually all vertebrate animals with important roles in regard to red blood cells and the immune system. In humans, it is located in the left upper quadrant of the abdomen. It removes old red blood cells and holds a reserve of blood in case of hemorrhagic shock...
but this is very rare.
Diagnosis is made with a biopsy of affected tissue, this shows the characteristic helm-shaped yeasts and culture shows the agent. Serology is also used in endemic areas.
Treatment
SulfonamidesSulfonamide (medicine)
Sulfonamide or sulphonamide is the basis of several groups of drugs. The original antibacterial sulfonamides are synthetic antimicrobial agents that contain the sulfonamide group. Some sulfonamides are also devoid of antibacterial activity, e.g., the anticonvulsant sultiame...
are the traditional remedies to paracoccidiodomycosis. They were introduced by Oliveira Ribeiro and used for more than fifty years with good results. The most used sulfa drugs in this infection are sulfadimethoxime, sulfadiazine
Sulfadiazine
Sulfadiazine is a sulfonamide antibiotic.-Uses:It eliminates bacteria that cause infections by stopping the production of folic acid inside the bacterial cell, and is commonly used to treat urinary tract infections ....
and co-trimoxazole
Co-trimoxazole
Trimethoprim/sulfamethoxazole or co-trimoxazole is a sulfonamide antibiotic combination of trimethoprim and sulfamethoxazole, in the ratio of 1 to 5, used in the treatment of a variety of bacterial infections.The name co-trimoxazole is the British Approved Name, and has been marketed worldwide...
. This treatment is generally safe but several adverse effects can appear, the most severe of which are the Stevens Johnson Syndrome and agranulocytosis
Agranulocytosis
Granulopenia, also known as Agranulosis or Agranulocytosis, is an acute condition involving a severe and dangerous leukopenia , most commonly of neutrophils causing a neutropenia in the circulating blood. It represents a severe lack of one major class of infection-fighting white blood cells...
. Similarly to tuberculosis
Tuberculosis
Tuberculosis, MTB, or TB is a common, and in many cases lethal, infectious disease caused by various strains of mycobacteria, usually Mycobacterium tuberculosis. Tuberculosis usually attacks the lungs but can also affect other parts of the body...
treatment, it must be continued for up to 3 years to eradicate the fungus, and relapse and treatment failures aren't unusual.
Antifungal drugs like amphotericin B
Amphotericin B
Amphotericin B is a polyene antifungal drug, often used intravenously for systemic fungal infections...
or ketoconazole
Ketoconazole
Ketoconazole is a synthetic antifungal drug used to prevent and treat fungal skin infections, especially in immunocompromised patients such as those with AIDS or those on chemotherapy. Ketoconazole is sold commercially as an anti-dandruff shampoo, topical cream, and oral tablet.Ketoconazole is...
are also effective in clearing the infection but they are very expensive compared with sulfonamides.
During therapy fibrosis
Fibrosis
Fibrosis is the formation of excess fibrous connective tissue in an organ or tissue in a reparative or reactive process. This is as opposed to formation of fibrous tissue as a normal constituent of an organ or tissue...
can appear and a surgery be needed to correct this. Other possible complication is Addisonian crisis. The death rate is approximately ten percent.
Discovery
Lutz-Splendore-de Almeida disease is named for the physicians Adolfo LutzAdolfo Lutz
Adolfo Lutz was a Brazilian physician, 1855-1940, father of tropical medicine and medical zoology in Brazil, and a pioneer epidemiologist and researcher in infectious diseases....
, Alfonso Splendore, and Floriano Paulo de Almeida, who first characterized the disease in Brazil in the early 20th century.
External links
- Overview at University of AdelaideUniversity of AdelaideThe University of Adelaide is a public university located in Adelaide, South Australia. Established in 1874, it is the third oldest university in Australia...