$Unique_ID{BRK04083} $Pretitle{} $Title{Paracoccidioidomycosis} $Subject{Paracoccidioidomycosis South American Blastomycosis Lutz-Splendore-Almeida Disease Paracoccidioidal Granuloma Lobo Disease} $Volume{} $Log{} Copyright (C) 1986, 1988 National Organization for Rare Disorders, Inc. 159: Paracoccidioidomycosis ** IMPORTANT ** It is possible that the main title of the article (Paracoccidioidomycosis) is not the name you expected. Please check the SYNONYM listing to find the alternate names and disorder subdivisions covered by this article. Synonyms South American Blastomycosis Lutz-Splendore-Almeida Disease Paracoccidioidal Granuloma Including: Lobo Disease General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section. Paracoccidioidomycosis is an infectious fungal disease involving the skin, mucous membranes, lymph nodes, and internal organs. The disease occurs primarily in South and Central America. It usually affects men between the ages of 20 and 50 years. The disease is fatal if not treated, but several effective therapeutic drugs exist. Symptoms Paracoccidioidomycosis occurs in four clinical forms, depending on the affected individual's immunological reaction to the pathogenic organism, and the extent to which the organism becomes systemic. The four forms are: l. Mucocutaneous Paracoccidioidomycosis 2. Lymphatic Paracoccidioidomycosis 3. Visceral Paracoccidioidomycosis 4. Pulmonary Paracoccidioidomycosis The four forms may occur simultaneously, only one may occur, or one may give way to another as the disease progresses. The pathogen spreads from one part of the body to another primarily via the lymph and blood. Mucocutaneous Paracoccidioidomycosis affects the skin, mucous membranes, and the borders between the two, particularly on the face, mouth (including the gums, tongue, palate, tonsils), and nose. Small ulcers with tiny yellowish areas containing the fungus gradually expand over the area involved, while nearby lymph nodes enlarge and adhere to the undersurface of the skin causing some pain. As cells of the lymph nodes are destroyed, they discharge a puss-like fluid through the skin. In the lymphatic form of Paracoccidioidomycosis, larger lymph nodes are involved in addition to those described above. The nodes in the mesentery (membrane connecting and supporting the abdominal organs), the neck, and the armpits are greatly, although generally painlessly, enlarged. The spleen, liver, pancreas, kidneys, intestines, and rarely, the brain, are involved in Visceral Paracoccidiodomycosis. In Pulmonary Paracoccidioidomycosis, the patient has a cough, often with bloody sputum, difficulty breathing, fever, fatigue, weight loss, and an overall feeling of discomfort. Causes Paracoccidioidomycosis is caused by the fungus Paracoccidioides brasiliensis. (This fungus used to be known as Blastomyces brasiliensis.) Affected Population Paracoccidiodomycosis occurs only in Central and South America, and is especially prevalent in the coffee growing regions of Brazil. It affects men about 10 times as often as women. Most affected men are between 20 and 50 years of age. Related Disorders Paracoccidioidomycosis is a systemic fungal disease, of which there are many other examples. The organism causing this disorder used to be classified in the same group as that responsible for Blastomycosis. Thus, Blastomycosis was known as North American Blastomycosis, while Paracoccidioidomycosis was known as South American Blastomycosis. Therapies: Standard Amphotericin B is the antifungal treatment of choice for Paracoccidiodomycosis, and is usually effective. In patients who do not tolerate this drug, sulfonamides suppress symptoms and progress of the disease, but do not eliminate the pathogen. Therapies: Investigational Ketoconazole is currently under investigation for the treatment of Paracoccidiodomycosis and other systemic fungal infections. This disease entry is based upon medical information available through September 1989. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Paracoccidiomycosis, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 Centers for Disease Control (CDC) 1600 Clifton Road, NE Atlanta, GA 30333 (404) 639-3534 NIH/National Institute of Allergy and Infectious Diseases (NIAID) 9000 Rockville Pike Bethesda, MD 20892 (301) 496-5717 References CECIL TEXTBOOK OF MEDICINE, 18th ed.: James B. Wyngaarden, and Lloyd H. Smith, Jr., Eds.: W. B. Saunders Co., 1988. Pp. 1843-4. THE MERCK MANUAL 15th ed: R. Berkow, et al: eds; Merck, Sharp & Dohme Research Laboratories, 1987. P. 143.