Toxic Shock Syndrome
Toxic Shock Syndrome
National Organization for Rare Disorders, Inc.
It is possible that the main title of the report Toxic Shock Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
Related Disorders List
Information on the following diseases can be found in the Related Disorders section of this report:
- Streptococcal Toxic Shock Syndrome
- Scarlet Fever
- Kawasaki Disease
- Associated Disorders (General)
Toxic Shock Syndrome is a rare multisystem disease with many widespread symptoms. It is caused by a toxin that is produced and secreted by the bacterium Staphylococcus aureus. The symptoms of Toxic Shock Syndrome may include a sudden high fever, nausea, vomiting, diarrhea, abnormally low blood pressure (hypotension), and a characteristic skin rash that resemble a bad sunburn. Most cases of Toxic Shock Syndrome occur in menstruating females in association with the use of tampons. Other cases may occur in association with postoperative wound infections, nasal packing, or other factors.
The symptoms of Toxic Shock Syndrome begin suddenly and usually include high fever, headache, sore throat (pharyngitis), inflammation of the whites of the eyes (conjunctivitis), muscle aches and pain (myalgia), and/or certain digestive symptoms, such as nausea, vomiting, and profuse watery diarrhea. Involvement of the central nervous system is also common and may be characterized by listlessness, dizziness, confusion, and/or disorientation. A characteristic "sunburn-like" skin rash typically develops within a few hours of onset, with later scaling and peeling (desquamation) of skin, particularly of the palms and soles.
In severe cases, blood pressure may fall dangerously low (hypotension), with an inadequate blood supply to body tissues (shock). Additional complications may include kidney and/or liver failure, heart dysfunction, adult respiratory distress syndrome, and/or other abnormalities. Adult respiratory distress syndrome is characterized by shortness of breath (dyspnea), abnormally rapid breathing, and insufficient levels of oxygen in the circulating blood. Without early diagnosis and appropriate treatment of Toxic Shock Syndrome, potentially life-threatening complications may result.
Toxic Shock Syndrome (TSS) is a rare acute multisystemic disease caused by toxins (such as "toxic shock syndrome toxin-1" [TSST-1], enterotoxin B, enterotoxin C) produced by certain strains (particularly phage group I) of the bacterium Staphylococcus aureus (S. aureus). TSS is most common in menstruating women who use highly absorbent tampons. Evidence suggests that the prolonged use of tampons in the presence (i.e., colonization) of toxin-secreting S. aureus strains may promote increased production of the toxin, which may enter the bloodstream through the uterus or tiny cuts within the vaginal lining.
In addition, TSS may also occur in nonmenstruating women or those who do not use tampons. Such "nonmenstrual TSS" may occur due to vaginal colonization of toxin-secreting S. aureus strains and certain associated factors, such as the use of vaginal contraceptive devices (e.g., diaphragms, contraceptive sponges); vaginal infection; childbirth or the period (e.g., hours to several weeks) following delivery (postpartum state); or other factors. Nonmenstrual TSS may also occur in women, men, or children in association with post-surgical wounds; nasal packing; burns; certain skin infections; other inflammatory conditions, such as of the windpipe (tracheitis), the sinuses (sinusitis), the lungs (pneumonia), or the bone or bone marrow (osteomyelitis); the "flu" (influenza); or other factors.
Toxic Shock Syndrome (TSS) was originally described as a disease entity in 1978, when the condition was reported in seven children from age eight to 17 years. In 1980 and 1981, a large number of TSS cases were recognized, primarily in young menstruating women who used tampons. This sudden increase was thought to be due to the introduction and availability of new, highly absorbent tampons.
Since the removal of super absorbent tampon varieties from the market and the introduction of federal regulations, the incidence of TSS has declined in women in the United States. (Incidence refers to the number of new cases of a particular disorder or condition during a specific period.) For example, in 1980 in the U.S., the incidence of TSS was approximately six per 100,000 women aged 19 to 44 years. In 1986 and later, the incidence decreased among this age group to one per 100,000. Other estimates suggest that TSS may currently occur in up to three per 100,000 menstruating women. Investigators indicate that menstruation-associated TSS most commonly occurs in young women aged 15 to 25 years who are using tampons.
As noted above, TSS may also occur in nonmenstruating women and in women who do not use tampons as well as in men and children. Nonmenstrual TSS more commonly affects females than males by a ratio of about three to one. (For further information, please see the "Causes" section above.)
Symptoms of the following disorders may be similar to those of Toxic Shock Syndrome (TSS). Comparisons may be useful for a differential diagnosis:
Streptococcal Toxic Shock Syndrome may be characterized by TSS-like symptoms and findings, the sudden onset of shock, and impairment of various organ systems due to infection with certain toxin-secreting strains of streptococcus bacteria (group A beta-hemolytic streptococci). The syndrome, which was initially reported in the mid to late 1980s, may occur in adults or children. The skin and soft tissues are usually the primary sites of infection. Factors that appear to increase the risk of infection may include certain surgical procedures, particular viral infections (e.g., varicella, influenza), and exposure to affected individuals in close environments such as hospitals or nursing homes. Associated symptoms and findings may vary from case to case, depending upon the causative bacterial strain and other factors. Reported features have included initial flu-like symptoms, including fever, chills, muscle aches, and vomiting; low blood pressure (hypotension); confusion; an accelerated heart rate (tachycardia), unusually rapid breathing (tachypnea), and other findings; and sudden onset of shock and multiple organ failure, leading to potentially life-threatening complications.
Scarlet Fever is an infectious disease of childhood usually caused by certain toxin-secreting strains of streptococci bacteria (group A beta-hemolytic streptococci). Symptoms may include sore throat, fever, chills, swollen lymph nodes in the neck, a strawberry color to the tongue, and the development of a pinkish-red rash that may be most apparent on the sides of the chest, on the abdomen, and within skin folds. Following resolution of fever, the outer region of previously reddened skin may peel. Other symptoms and findings may also be present.
Kawasaki Disease is a rare inflammatory disease of unknown cause that most commonly affects infants and young children. It may be characterized by a sudden fever that lasts for approximately one week or longer; dryness and cracking of the lips; a strawberry-red tongue; swelling of one or more lymph nodes (lymphadenopathy); and/or a reddish skin rash affecting the hands and feet, trunk, and other regions. By about two to three weeks following symptom onset, affected skin tissue may peel (desquamate) from the tips of the fingers and toes, palms of the hands, soles of the feet, and/or other affected areas. Other symptoms and findings may include inflammation and redness of the "whites" of the eyes (bilateral conjunctivitis); irritability; fatigue; inflammation, pain, and swelling of many joints (polyarthritis); and/or other abnormalities. In addition, affected individuals may develop inflammation of arteries that transport blood to heart muscle (coronary arteritis); associated bulging or widening (aneurysms) of the walls of affected coronary arteries; inflammation of heart muscle (myocarditis); and/or other symptoms and findings. Evidence suggests that Kawasaki Disease is the primary cause of acquired heart disease in children in the United States. Some researchers indicate that toxic substances produced by certain forms of bacteria, such as streptococci or staphylococci, may play some role in causing the inflammatory disease process. (For more information on this disorder, choose "Kawasaki" as your search term in the Rare Disease Database.)
The Ehrlichioses are rare infectious diseases caused by bacteria in the Ehrlichia family. Three forms of Human Ehrlichial infection have been identified, including Human Granulocytic Ehrlichiosis (HGE), Human Monocytic Ehrlichiosis (HME), and Sennetsu Fever. The bacteria that cause HGE and HME are carried and transmitted by certain ticks. Cases of Sennetsu Fever, which have been limited to Malaysia and Western Japan, are thought to result from ingestion of raw fish. Though caused by different strains of Ehrlichia bacteria, the three disorders are characterized by similar symptoms. Such symptoms may include a sudden high fever, headache, muscle aches (myalgia), chills, a general feeling of ill health (malaise), and fatigue. Some affected individuals may also develop nausea, vomiting, sore throat, cough, diarrhea, loss of appetite, weight loss, and/or other symptoms. Rarely, a skin rash may appear. Laboratory testing may reveal abnormally low numbers of circulating blood platelets (thrombocytopenia), reduced numbers of white blood cells (leukopenia), and increased levels of certain liver enzymes. Without prompt, appropriate treatment, potentially life-threatening complications may occur in some cases. (For further information, choose "Ehrlichiosis" as your search term in the Rare Disease Database.)
Leptospirosis is a group of infectious diseases caused by bacterial organisms known as spirochetes (Leptospira), which are transmitted in the urine of certain domestic and wild animals, including dogs, raccoons, and rats. Symptoms may include fever; chills; muscle aches; inflammation of the protective membranes covering the brain (meningitis), with associated headache, stiffness of the neck, and, in some cases, mental changes; and/or other abnormalities. The most severe form of Leptospirosis, known as Weil Syndrome, may also be characterized by reduced blood clotting and associated bleeding within tissues; inflammation of the liver (hepatitis); yellowish discoloration of the skin, mucous membranes, and whites of the eyes (jaundice); kidney (renal) failure; and/or other complications. (For more information on this disorder, choose "Leptospirosis" as your search term in the Rare Disease Database.)
Other infectious diseases or inflammatory syndromes may potentially cause certain symptoms and findings similar to those associated with Toxic Shock Syndrome. (For further information, please choose the exact disease name in question as your search term in the Rare Disease Database.)
The diagnosis of Toxic Shock Syndrome (TSS) has been defined by the Centers for Disease Control clinical and laboratory criteria. TSS is considered probable if three or more criteria are met in association with peeling (desquamation) of affected skin or if five or more criteria are met in the absence of desquamation. The criteria include the following: fever; rash, with possible, subsequent peeling (desquamation), particularly on the palms and soles; low blood pressure (hypotension); the involvement of three or more organ systems (i.e., digestive [gastrointestinal], muscular, mucous membranes, kidneys, liver, blood, and/or brain and spinal cord [central nervous system]), and negative results of blood tests for the infectious diseases Rocky Mountain Spotted Fever, Leptospirosis, and Measles. Sometimes S. aureus may be isolated from the vagina or from localized (focal) wound sites and identified with the use of various laboratory techniques (e.g., bacterial cultures).
Individuals with Toxic Shock Syndrome (TSS) should immediately be hospitalized for appropriate intensive treatment. Disease management requires aggressive intravenous fluid and electrolyte replacement and other supportive care measures as appropriate. Vaginal examination is also essential in women to remove a tampon or vaginal contraceptive device and to collect small tissue samples (cultures) from the vagina and neck of the uterus (cervix) for possible isolation of the S. aureus bacterium. In nonmenstrual TSS, measures may include obtaining cultures from and appropriately cleaning and treating focal wound sites.
In addition, in individuals with TSS, treatment typically includes the intravenous administration of beta-lactamase resistant antistaphylococcal antibiotics, such as nafcillin or oxacillin, possibly in combination with clindamycin. Other appropriate antibiotic regimens may be required in some cases. Such antibiotic therapy is important in preventing recurrences of TSS. In extremely severe cases, treatment may include infusion of concentrated preparations of certain antibodies (intravenous immune globulins).
To prevent menstrual TSS, tampons should only be used intermittently and replaced frequently. Super absorbent brands should also be avoided. Women who use tampons should be aware of the symptoms of TSS and advised to seek immediate medical attention if they occur. In addition, avoidance of tampons is important in helping to prevent a recurrence of menstrual TSS.
As noted above (see "Causes"), TSS has also been reported in association with the use of certain vaginal contraceptive devices. Women who use such contraceptive methods are cautioned to carefully follow all package instructions and to share any questions and concerns with their physicians and pharmacists to help minimize risk and ensure proper use.
Other treatment for this disorder is symptomatic and supportive.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. Government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
For information about clinical trials sponsored by private sources, contact:
Mandell GL, et al., eds. Mandell, Douglas, and Bennett's Principles and Practice of Infectious Diseases. 5th ed. Philadelphia, PA: Churchill Livingstone; 2000:2080-82, 2110-13.
Wyngaarden JB, et al., eds. Cecil Textbook of Medicine. 19th ed. Philadelphia, PA: W.B. Saunders Company; 1992:780, 1629, 1650, 2301-02.
Beers MH, et al., eds. The Merck Manual. 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:1149-50, 1152.
Behrman RE, et al., eds. Nelson Textbook of Pediatrics. 15th ed. Philadelphia, PA: W.B. Saunders Company; 1996:749-50.
Baxter F, et al. Severe group A streptococcal infection and streptococcal toxic shock syndrome. Can J Anaesth. 2000;47:1129-40.
Givner LB. Invasive disease due to group A beta-hemolytic streptococci: continued occurrence in children in North Carolina. South Med J. 1998;91:333-37.
Fishman G, et al. Toxic shock syndrome. Harefuah. 1997;132:622-24, 679.
Fichtenbaum CJ, et al. Ehrlichiosis presenting as a life-threatening illness with features of the toxic shock syndrome. Am J Med. 1993;95:351-57.
Strausbaugh LJ. Toxic shock syndrome. Are you recognizing its changing presentations? Postgrad Med. 1993;94:107-08, 111-13, 117-18.
Kain KC, et al. Clinical spectrum of nonmenstrual toxic shock syndrome (TSS): comparison with menstrual TSS by multivariate discriminant analyses. Clin Infect Dis. 1993;16:100-06.
Panina-Bordignon P, et al. Identification of HLA-DR alpha chain residues critical for binding of the toxic shock syndrome toxin superantigen. J Exp Med. 1992;176:1779-84.
McCahill PD, et al. Toxic shock syndrome: a complication of continent urinary diversion. J Urol. 1992;147:681-82.
Schuchat A, et al. Toxic shock syndrome and tampons. Epidemiol Rev. 1991;13:99-112.
Givner LB, et al. Apparent increase in the incidence of invasive group A beta- hemolytic streptococcal disease in children. J Pediatr. 1991;18:341-46.
Todd JK. Therapy of toxic shock syndrome. Drugs. 1990;39:856-61.
Allen ST, et al. Toxic shock syndrome associated with use of latex nasal packing. Arch Intern Med. 1990;150:2587-88.
Bryner CL, et al. Recurrent toxic shock syndrome. Am Fam Physician. 1989;39:157-64.
Rizkallah MF, et al. Toxic shock syndrome caused by a strain of staphylococcus aureus that produces enterotoxin C but not toxic shock syndrome toxin-1. Am J Dis Child. 1989; 143:848-49.
Prechter GC, et al. Postinfluenza toxic shock syndrome. Chest. 1989;95:1153-54.
Kass EH, et al. On the pathogenesis of toxic shock syndrome. Rev Infect Dis. 1987;9:S482-89.
Osterholm, MT, et al. Tri-state toxic-shock syndrome study. I. Epidemiologic findings. J Infect Dis. 1982;145:431-40.
Davis JP, et al. Toxic-shock syndrome: epidemiologic features, recurrence, risk factors, and prevention. New Engl J Med. 1980;303:1429-35.
Todd J, et al. Toxic-shock syndrome associated with phage-group-I staphylococci. Lancet. 1978;2:1116-18.
Centers for Disease Control and Prevention
1600 Clifton Road NE
Atlanta, GA 30333
NIH/National Institute of Allergy and Infectious Diseases
NIAID Office of Communications and Government Relations
5601 Fishers Lane, MSC 9806
Bethesda, MD 20892-9806
World Health Organization (WHO)
Avenue Appia 20
Geneva 27, 1211
Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
For a Complete Report
This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.
The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.
It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report
This disease entry is based upon medical information available through the date at the end of the topic. 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.
For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email firstname.lastname@example.org
Last Updated: 4/8/2009
Copyright 1986, 1992, 1994, 2001, 2002, 2009 National Organization for Rare Disorders, Inc.
Healthwise, Healthwise for every health decision, and the Healthwise logo are trademarks of Healthwise, Incorporated.