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Disease Profile

Staphylococcal toxic shock syndrome

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
1-9 / 100 000

3,310 - 29,790

US Estimated

1-9 / 100 000

5,135 - 46,215

Europe Estimated

Age of onset

All ages

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ICD-10

A48.3

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Bacterial toxic-shock syndrome

Summary

The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs.
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Orpha Number: 36234

Definition
Bacterial toxic shock syndrome (TSS) is a potentially fatal, acute disease characterized by a sudden onset of high fever along with nausea, myalgia, vomiting and multisystem organ involvement, potentially leading to shock and death. TSS is mediated by superantigenic toxins, usually caused by an infection with Staphylococcus aureus in staphylococcal TSS (see this term) or Streptococcus pyogenes in streptococcal TSS (see this term).

Epidemiology
The worldwide prevalence is estimated at 1/30, 000.

Clinical description
The disease affects mainly young adult women with no previous medical conditions. However, non-menstrual TSS, which can also be observed in men and children, represents up to 50% of Staphylococcal TSS cases. Onset is sudden and includes high fever (>38.9°C), nausea, diarrhea, vomiting, myalgia, abdominal pain and sore throat. Staphylococcal TSS almost always exhibits an erythematous rash and skin peeling as one of its manifestations, whereas it is very rarely seen in streptococcal TSS, which can show signs of soft tissue infection. Serious manifestations include confusion, shock, renal and myocardial dysfunction, acute respiratory distress syndrome (ARDS; see this term) and coma.

Etiology
Staphylococcal TSS is due to an infection with Staphylococcus aureus and streptococcal TSS is due to an infection with Streptococcus pyogenes, or rarely, group C or G Streptococcus. Superantigens produced by both bacteria are responsible for the massive and sudden immune reaction seen in TSS. Both forms have been associated with recent traumas (surgery or childbirth) and viral infections, while staphylococcal TSS has also been associated with high absorbency tampon use.

Visit the Orphanet disease page for more resources.

Symptoms

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Hypotension
Low blood pressure
0002615
Shock
0031273
30%-79% of people have these symptoms
Abdominal pain
Pain in stomach
Stomach pain

[ more ]

0002027
Abnormality of facial soft tissue
Anomaly of facial soft tissue
0011799
Confusion
Disorientation
Easily confused
Mental disorientation

[ more ]

0001289
Fasciitis
Inflammation of the fascia
0100537
Fever
0001945
Increased serum lactate
0002151
Metabolic acidosis
0001942
Myalgia
Muscle ache
Muscle pain

[ more ]

0003326
Recurrent skin infections
Skin infections, recurrent
0001581
Respiratory distress
Breathing difficulties
Difficulty breathing

[ more ]

0002098
Tachycardia
Fast heart rate
Heart racing
Racing heart

[ more ]

0001649
5%-29% of people have these symptoms
Abnormal blistering of the skin
Blistering, generalized
Blisters

[ more ]

0008066
Abnormality of the lower limb
Lower limb deformities
0002814
Abnormality of the upper limb
0002817
Abscess
0025615
Acute cutaneous wound
0032675
Bacteremia
0031864
Capillary leak
0030005
Cellulitis
Bacterial infection of skin
Skin infection

[ more ]

0100658
Chills
0025143
Diarrhea
Watery stool
0002014
Disseminated intravascular coagulation
0005521
Ecchymosis
0031364
Elevated serum creatine kinase
Elevated blood creatine phosphokinase
Elevated circulating creatine phosphokinase
Elevated creatine kinase
Elevated serum CPK
Elevated serum creatine phosphokinase
High serum creatine kinase
Increased CPK
Increased creatine kinase
Increased creatine phosphokinase
Increased serum CK
Increased serum creatine kinase
Increased serum creatine phosphokinase

[ more ]

0003236
Elevated serum creatinine
Elevated creatinine
High blood creatinine level
Increased creatinine
Increased serum creatinine

[ more ]

0003259
Encephalitis
Brain inflammation
0002383
Glomerulonephritis
0000099
Hepatitis
Liver inflammation
0012115
Hypoalbuminemia
Low blood albumin
0003073
Hypocalcemia
Low blood calcium levels
0002901
Increased circulating metamyelocyte count
0032238
Increased circulating myelocyte count
0032237
Localized skin lesion
0011355
Meningitis
0001287
Myocarditis
Inflammation of heart muscle
0012819
Myositis
Muscle inflammation
0100614
Nausea
0002018
Osteomyelitis
Bone infection
0002754
Peritonitis
0002586
Pharyngitis
0025439
Pneumonia
0002090
Renal insufficiency
Renal failure
Renal failure in adulthood

[ more ]

0000083
Scaling skin
flaking skin
peeling skin
scaly skin

[ more ]

0040189
Sepsis
Infection in blood stream
0100806
Septic arthritis
0003095
Severe varicella zoster infection
0032170
Sinusitis
Sinus inflammation
0000246
Skin rash
0000988
Tachypnea
Increased respiratory rate or depth of breathing
0002789
Thrombocytopenia
Low platelet count
0001873
Vomiting
Throwing up
0002013
1%-4% of people have these symptoms
Edema
Fluid retention
Water retention

[ more ]

0000969
Recurrent urinary tract infections
Frequent urinary tract infections
Repeated bladder infections
Repeated urinary tract infections
Urinary tract infections
Urinary tract infections, recurrent

[ more ]

0000010

Learn more

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

In-Depth Information

  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.