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$Unique_ID{BRK03929} $Pretitle{} $Title{Leprosy} $Subject{Leprosy Hansen's Disease Lepra Indeterminate Leprosy Tuberculoid Leprosy (Minor and Major) Benign Type Hansen's Disease Lepromatous Leprosy (Malignant Type Hansen's Disease) Dimorphous Leprosy (Borderline Leprosy) Mycosis Fungoides Lymphocytic Infiltrate of Jessner Lupus Miliaris Disseminatus Faciei Lupus Vulgaris} $Volume{} $Log{} Copyright (C) 1986, 1990, 1992, 1993 National Organization for Rare Disorders, Inc. 67: Leprosy ** IMPORTANT ** It is possible that the main title of the article (Leprosy) is not the name you expected. Please check the SYNONYMS listing to find the alternate name and disorder subdivisions covered by this article. Synonyms Hansen's Disease Lepra Disorder Subdivisions: Indeterminate Leprosy Tuberculoid Leprosy (Minor and Major) Benign Type Hansen's Disease Lepromatous Leprosy (Malignant Type Hansen's Disease) Dimorphous Leprosy (Borderline Leprosy) Information on the following diseases can be found in the Related Disorders section of this report: Mycosis Fungoides Lymphocytic Infiltrate of Jessner Lupus Miliaris Disseminatus Faciei Lupus Vulgaris 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 of this report. Leprosy is a progressive, chronic infectious disease caused by the bacteria, Mycobacterium leprae. This disease affects the nerves that are located outside the central nervous system (peripheral nerves), and the skin, mucous membranes, and eyes. In severe cases of Leprosy, loss of sensation, disfigurement, and/or blindness may occur. There are several forms of Leprosy: Tuberculoid (Minor and Major, and Benign Hansen's Disease), Lepromatous (Malignant Hansen's Disease), Dimorphous (Borderline Leprosy), and Indeterminate Leprosy. This disease is rare in the United States but prevalent in third world countries. Symptoms Leprosy is a slowly progressive infectious disease that affects nerves and the skin of the face, hands, lower legs, and/or feet. The symptoms of nerve involvement include burning and tingling sensations (paresthesias), a lack of sensation or feeling in the affected areas (anesthesia), weakness, paralysis, and/or the loss of muscle tissue (atrophy). Nerve lesions tend to occur in the skin and along the areas where nerves are grouped together (nerve trunks). Skin lesions include flat, spotty discolorations (macules), raised areas of red skin (papules), small solid masses (nodules) and/or raised discolorations (plaques). Plaques may be reddish (erythematous) or pale (hypopigmented). Leprosy is classified into the subtypes Tuberculoid and Lepromatous, and there are intermediate subtypes between these two. Tuberculoid Leprosy is the mildest subtype of the disease and is characterized by large plaques that are dry, hairless, and/or numb to the touch (anesthetic). The intermediate or borderline subtype of Leprosy is more severe and is characterized by numerous skin lesions but less loss of feeling. The symptoms of people with Intermediate or Borderline Leprosy tend to progress over time toward either the Tuberculoid or the Lepromatous form of the disease. People with Lepromatous Leprosy have less severe loss of feeling. However, involvement of the skin and nerves is more extensive and invasive. Solid nodular skin lesions are characteristic of this subtype of the disease. The complications that may occur include eye involvement and deformities of the face, hands, and/or feet. Deformities of the face can result from destruction by the bacteria, causing deterioration of the partition in the nose that divides the nostrils (nasal septum), cartilage, and other facial tissues. People with Lepromatous Leprosy usually lose their eyebrows and eyelashes, and the earlobes may enlarge. Deformities of the hands and feet may result from repeated trauma that is not felt due to sensory loss. Eye damage and visual impairment may occur in people with Leprosy. Symptoms may include inflammation of the membranes that line the eyes (conjunctivitis) and the corneas of the eyes (keratitis). Inflammation of the iris of the eyes (iridocyclitis) may lead to the loss of transparency in the lens of the eyes (cataracts). A lack of feeling in the cornea of the eyes (corneal anesthesia) may result from involvement of the nerves in the face (trigeminal nerve) and result in damage to the corneas and possibly blindness. Another serious complication of Lepromatous Leprosy is Erythema Nodosum Leprosum (ENL). This syndrome is characterized by high fevers, the decay (necrosis) of skin nodules, and pain as a result of inflammation of nerves. Erythema Nodosum Leprosum may also be associated with joint disease (polyarthralgia) and inflammation of the nerve fibers within the kidneys (glomerulonephritis). Some patients with Intermediate or Tuberculoid Leprosy experience a worsening of disease while on therapy (reversal reaction). This appears to be the result of a reaction of the immune system. If this occurs, the skin lesions may become deeper and may ulcerate. Other neurological symptoms may also worsen. The abnormal accumulation of a fatty substance in different parts of the body (Amyloidosis) is a complication of Leprosy. The prevalence of this disorder in association with Leprosy varies from one geographic region to another. The characteristic lesion of Leprosy is a mass of nodular granulated tissue (granuloma). These may appear on the skin, lymph nodes, liver, and/or spleen. The diagnosis of this disorder is made by skin biopsy, and it is important to examine a large specimen to be certain of the diagnosis of Leprosy. A biopsy that removes tissue from the affected area (excisional biopsy) is preferred to a small punch biopsy. Causes Leprosy is caused by the bacteria Mycobacterium leprae. These bacteria thrive in an acid environment and can only grow in living hosts. The way in which Leprosy is transmitted is not fully understood. This disease may possibly spread by direct skin contact, by inhalation, or by sexual contact. However, prolonged exposure to the person affected with Leprosy is necessary. Breast feeding and transmission by insects have also been implicated as a possible mode of transmission for this disease. Affected Population Leprosy affects approximately 12 million to 15 million people worldwide. Children are more susceptible to infection than adults. Leprosy is a major problem in tropical regions of Asia, Africa, and South America. It is also prevalent in some islands of the South Pacific. In the rare cases of Leprosy in the United States, they occur in the southern most parts of the country (i.e., around the Gulf of Mexico), but most of the cases appear in people who have immigrated from other countries. North American Indians appear to be immune to this disease. The number of cases of Leprosy is currently rising in the United States, due to large numbers of immigrants from regions where this disease is more common, particularly Southeast Asia. Approximately 8,000 cases had been identified in the United States in 1989, and were treated in U.S. Public Health Service Hospitals. Related Disorders Symptoms of the following disorders can be similar to those of Leprosy. Comparisons may be useful for a differential diagnosis: Mycosis Fungoides is a chronic progressive disorder of lymphocytes characterized by a red skin rash or psoriatic patches of dry blisters. The affected areas are very red and painful. These scattered red patches may appear on the trunk of the body, or on the arms and legs. As the disease progresses, lesions on the skin typically become bluish-red and elevated. Other symptoms may include anemia, weight loss, fever, and digestive difficulties. (For more information on this disorder, choose "Mycosis Fungoides" as your search term in the Rare Disease Database.) Lymphocytic Infiltrate of Jessner is a rare blood disorder characterized by benign solid lesions of the skin that may appear on the neck, face and/or back. The lesions are typically smooth and pink or red, have no hair follicles, and are sometimes clear in the center. The skin that surrounds the lesions is usually swollen and red. After several years, the lesions generally disappear. (For more information on this disorder, choose "Lymphocytic Infiltrate of Jessner" as your search term in the Rare Disease Database.) Lupus Miliaris Disseminatus Faciei is a chronic skin infection caused by Mycobacterium Tuberculosis. It is characterized by soft, brownish-red papules that may appear alone or in clusters. Papules may appear on the face, neck, mouth, and/or nose. Healing is usually slow and scarring is common. Lupus Vulgaris, another form of Tuberculosis that affects the skin, is a progressive infection that may cause scarring and deformities of the face. The yellowish-brown lesions are typically small and soft; they may appear as crusted ulcers. Lupus Vulgaris is more common in children and young adults. Therapies: Standard Leprosy can be cured if treatment begins early. Treatment may include the use of drugs such as diaminodiphenylsulfone, dapsone, rifampin, and ethionamide. The drug Dapsone is a standard treatment for this disease, but it does not kill the bacteria that cause Leprosy; it simply stops the bacteria from reproducing. The orphan drug clofazimine (Lamprene) is now approved as a treatment for people with Leprosy who are resistant to Dapsone. The drug is manufactured by Ciba-Geigy Pharmaceuticals. A therapy that combines these drugs is currently recommended for the treatment of Leprosy. A typical drug regimen would include dapsone, rifampin, and clofazimine. Therapy must be continued for 2 years in all cases, and longer in some. With combination drug therapy, the relapse rate is less than 10 percent. Reversal reactions and Erythema Nodosum Leprosum (ENL) must be treated immediately. In cases of reactions, corticosteroid drugs are initially administered. If the reaction persists, clofamizine may be prescribed. Moderate reactions have also been treated with potassium antimony tartrate (tartar ametic), stibophen, or chloroquine. Specific therapy for the disease should be maintained throughout the reactive episode unless the patient fails to respond to treatment, or serious side effects appear. Corneal dryness is treated with eye drops and ophthalmic mucin substitutes. Ocular complications of Erythema Nodosum Leprosum (ENL) must be treated promptly to prevent permanent damage to the eyes. Local atropine and hydrocortisone eye drops may be used to keep the pupils dilated and reduce the inflammation until the reaction subsides. Supportive care is important. Any area that has lost sensation such as arms, legs, and eyes must be protected from injury that could lead to infection, mutilation, or blindness. Some people with Leprosy may benefit from special shoes that compensate for foot deformities. Surgery may be necessary to treat eye problems or correct certain deformities of the hands and feet. The goal of surgery is to help improve function and improve the quality of life. Therapies: Investigational At the present time, studies are being conducted on the effectiveness of the drugs solasulphone and acedapsone for the treatment of Leprosy. Vaccines are also being studied. Progress is hampered because it is not possible to culture and grow the bacteria in the laboratory (in vitro). The orphan drug thalidomide is also being tested for use as a treatment for Leprosy. This drug is not used in women of childbearing age due to the possibility of serious birth defects. For more information on Thalidomide, physicians may contact: Pediatric Pharmaceuticals 379 Thornall Street Edison, NJ 08837 Thalidomide is available in England under special license from Penn Pharmaceuticals of Tredegar, South Wales. Research into the contagious aspects of Leprosy from person to person is ongoing because the mode of transmission is not understood. Since armadillos get a more serious infection than humans, scientists are trying to develop a vaccine from purified bacteria taken from armadillos. Another vaccine from monkeys is also being tested. This disease entry is based upon medical information available through May 1993. 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 Leprosy, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 National Hansen's Disease Center United States Public Health Service Hospital Carville, LA 70721 NIH/National Institute of Allergy and Infectious Diseases (NIAID) 9000 Rockville Pike Bethesda, MD 20892 (301) 496-5717 Centers for Disease Control (CDC) 1600 Clifton Road, NE Atlanta, GA 30333 (404) 639-3534 References CECIL TEXTBOOK OF MEDICINE, 19th Ed.: James B. Wyngaarden, and Lloyd H. Smith, Jr., Editors; W.B. Saunders Co., 1990. Pp. 1745-1751. THE MERCK MANUAL, 16th Ed.: Robert Berkow Ed.; Merck Research Laboratories, 1992. P. 146. PRINCIPLES OF NEUROLOGY, 4th Ed.; Raymond D. Adams, M.D. and Maurice Victor, M.D., Editors; McGraw-Hill Information Services Company, 1989. P. 1054. INSIDE THE SKIN: THE LOCAL IMMUNE AND INFLAMMATORY MILIEU IN LEPROSY. D.M. Scollard; Am J Trop Med Hyg (April 1991; 44(4.2). Pp. 17-23. LEPROSY. W.M. Meyers; Dermatol Clin (Jan. 1992; 10(1)). Pp. 73-96. CLOFAZIMINE: A REVIEW OF ITS USE IN LEPROSY AND MYCOBACTERIUM AVIUM COMPLEX INFECTION. J.C. Garrelts; DICP (May 1991; 25(5)). P. 5.