$Unique_ID{BRK03997} $Pretitle{} $Title{Meningococcemia} $Subject{Meningococcemia Meningococcal Disease Meningococcemia-Meningitis Fulminant Meningococcemia Waterhouse-Friderichsen Syndrome Chronic Meningococcemia Rocky Mountain Spotted Fever Henoch-Schonlein Purpura Acute Vasculitis Rheumatic Fever Toxic Shock Syndrome Bacterial Endocarditis} $Volume{} $Log{} Copyright (C) 1992 National Organization for Rare Disorders, Inc. 916: Meningococcemia ** IMPORTANT ** It is possible that the main title of the article (Meningococcemia) is not the name you expected. Please check the SYNONYM listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Meningococcal Disease Meningococcemia-Meningitis Disorder Subdivisions: Fulminant Meningococcemia (also known as Waterhouse-Friderichsen Syndrome) Chronic Meningococcemia Information on the following diseases can be found in the Related Disorders section of this report: Rocky Mountain Spotted Fever Henoch-Schonlein Purpura Acute Vasculitis Rheumatic Fever Toxic Shock Syndrome Bacterial Endocarditis 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. Meningococcemia is an infectious disease that occurs rarely. However, there are years when epidemics of the illness occur. Major symptoms may include upper respiratory tract infection, fever, skin rash and lesions, eye and ear problems and possibly shock (a sudden state of extreme physical depression which could cause a life-threatening situation). Symptoms Meningococcemia is characterized by sudden intense headache, nausea, fever, vomiting, skin rash and in cases associated with meningitis, a stiff neck. The patient may first complain of an upper respiratory infection. Chills may develop, then skin rash on the arms or legs and the trunk. Diarrhea may also be present. Later the rash may become widespread or develop into bleeding spots under the skin (petechiae, ecchymoses, or purpura). There may be associated swelling, muscle pain, skin deterioration or gangrene in the arms and legs. Pneumonia may also develop along with the other symptoms if the person has a suppressed immune system. In cases where meningitis occurs along with meningococcemia, the patient may have the symptoms listed above along with the combination of headache, confusion, stiff neck, and muscle pain from irritation of membranes surrounding the brain and spinal cord (meningismus). (For more information on this disorder, choose "Meningitis" as your search term in the Rare Disease Database). Disorder Subdivision Fulminant Meningococcemia is also known as Waterhouse-Friderichsen Syndrome and is the most severe form of the disorder. The disease comes on very suddenly and the progression of the symptoms is very rapid. In less than a few hours the patient has very high fever, chills, weakness, vomiting and severe headache. A red rash appears on the arms and legs and spreads very quickly over the body including the eyes and nose. The patient's blood pressure may drop dangerously and the fever may also drop dramatically. The patient may go into shock. Without immediate medical treatment this disorder can be life-threatening. Chronic Meningococcemia is a rarer form of the disease. It is characterized by fever that comes and goes over a period of weeks or months. Muscle and joint pain with headache as well as a skin rash may also come and go. This form of the disorder may also include an enlarged spleen. Causes Meningococcemia is caused by infection with the meningococci bacteria (Neisseria memingitidis) which are gram-negative diplococci bacteria. There are various groups of this bacteria that cause different forms of the disease and they are grouped by strains A,B,C,D,X,Y,Z, 29E and W135. These groups can be identified by testing the blood, scrapings of the skin rash and samples of the cerebrospinal fluid of the patient. Testing may take up to five days as the cultures are very slow growing. Infection with the bacteria is usually caused by a carrier. The natural place for the bacteria to be located is in either the nose or throat of the carrier, and they can be spread by airborne or close contact methods. The carrier may spread the infection for weeks or months if they are not diagnosed and treated. Affected Population Meningococcemia affects males and females in equal numbers. However, most cases develop in persons twenty years of age or younger and half of these cases are in children under five years of age. In the United States 1.2 cases per 100,000 occur annually. Winter and spring are the most common seasons of the year when cases are reported. Epidemics occur under crowded conditions and tend to occur at 20 to 30 year intervals. In other parts of the world epidemics are usually caused by the Group A strain of the bacteria. During epidemics, rates of 5 to 24 cases per 100,000 persons have occurred. In Sao Paulo, Brazil, during 1974 the epidemic rate was 370 per 100,000 persons infected with Meningococcemia. In the United States, the most prevalent Group strains of the bacteria are B,C,Y, and W135. Related Disorders Symptoms of the following disorders can be similar to those of Meningococcemia. Comparisons may be useful for a differential diagnosis: Rocky Mountain Spotted Fever is a tick-borne disease that begins with an incubation period of from two to twelve days. A gradually or abruptly beginning fever may be followed after three to five days by a pink or purplish colored rash on the wrists and ankles. The fever and rash usually become more severe for seven to fourteen days. The rash may not develop in all cases, possibly making diagnosis difficult. A blood test is necessary to confirm the diagnosis. (For more information on this disorder, choose "Rocky Mountain" as your search term in the Rare Disease Database). Shoenlein-Henoch Purpura is one of a group of disorders characterized by purplish or brownish red discolorations on the skin. These spots may be large or small. Internal bleeding may occur in various areas of the body. This blood vessel disorder may affect the skin, joints, gastrointestinal system, kidneys, and in a very few cases the central nervous system. (For more information on this disorder, choose "Shoenlein-Henoch" as your search term in the Rare Disease Database). Vasculitis is a common disorder characterized by an inflammation of the blood vessel walls. This inflammation causes a narrowing of the inside of the vessel and can obstruct the flow of blood to the tissues. Red or purple patches of discoloration may develop under the skin. Arteries and veins of all sizes and in all parts of the body may be affected. It may be localized or affect multiple areas of the body with inflammatory and destructive lesions. There may be muscle pain, joint pain, fever, weight loss and loss of appetite, headache, and generalized weakness. (For more information on this disorder, choose "Vasculitis" as your search term in the Rare Disease Database). Rheumatic Fever is an inflammatory syndrome that can occur following a streptococcal infection. Patients initially experience moderate fever, a general feeling of ill health, a sore throat, fatigue and a red rash. Major complications can include heart disease, joint pain and arthritis, involuntary abrupt limb movements with characteristic grimaces and skin symptoms. (For more information on this disorder, choose "Rheumatic" as your search term in the Rare Disease Database). Toxic Shock Syndrome symptoms appear very suddenly. Initially, there is a fever of 102 to 105 degrees F, headache, sore throat, and conjunctivitis. Other early symptoms include profound lethargy, periods of disorientation, vomiting, severe diarrhea, and a diffuse sunburn-like rash leading to sloughing of skin after several days. In severe cases, the syndrome may progress to shock (dangerously low blood pressure and circulatory collapse) within forty-eight hours. (For more information on this disorder, choose "Toxic Shock" as your search term in the Rare Disease Database). Infective Endocarditis usually has a very sudden onset. Complaints of low back pain, pain in the joints (arthralgia) or in one or more muscles (myalgia) are common. These symptoms usually appear early in the disease, occasionally as the only initial symptoms. Fever, night sweats, chills, headache and loss of appetite may also occur. Blood or blood cells may be present in the urine (hematuria), small red or purple spots composed of blood (petechiae) may cover the skin of the upper trunk and there may also be pale, oval spots on the retina of the eye. (For more information on this disorder, choose "Endocarditis" as your search term in the Rare Disease Database). Therapies: Standard Meningococcemia is usually treated with Penicillin or Ampicillin. In adults the method of treatment is often through intravenous Penicillin G. In children penicillin is still the treatment of choice, however, other organisms must be ruled out before treatment is begun. For persons who are unable to take penicillin, other antibiotics are used such as: cefuroxime, cefotaxime or ceftriaxone. In persons who survive severe meningococcal septicemia there may be ongoing problems with veins and arteries. There are usually serious orthopedic problems. If gangrene occurs amputation may be necessary. These patients should have continuing medical evaluations as a precaution against other conditions that can arise in later years. During times of epidemics, chemoprophylaxis (Rifampin) is used to protect persons exposed to or in close contact with infected patients. Therapies: Investigational This disease entry is based upon medical information available through May 1992. 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 Meningococcemia, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 NIH/National Institute of Allergy & 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. 1611-1617. CLINICAL DERMATOLOGY, 2nd Ed.; Thomas P. Habif, M.D., Editor: The C.V. Mosby Company, 1990. Pp. 210-211. CHONDRO-OSSEOUS GROWTH ABNORMALITIES AFTER MENINGOCOCCEMIA, A CLINICAL AND HISTOPATHOLOGICAL STUDY., Grogan, D.P., et al.; J Bone Joint Surg (AM), July, 1989, (issue 71 (6)). Pp. 920-928.