Find information on thousands of medical conditions and prescription drugs.

Stevens-Johnson syndrome

Stevens-Johnson syndrome (SJS) is a severe and potentially life-threatening (15% of cases) disease, it is a hypersensitivity complex affecting the skin and the mucous membranes, a severe expression of erythema multiforme (EM) (and so SJS is also called erythema multiforme major). more...

Home
Diseases
A
B
C
D
E
F
G
H
I
J
K
L
M
N
O
P
Q
R
S
Sabinas brittle hair...
Saccharopinuria
Sacral agenesis
Saethre-Chotzen syndrome
Salla disease
Salmonellosis
Sandhoff disease
Sanfilippo syndrome
Sarcoidosis
Say Meyer syndrome
Scabies
Scabiophobia
Scarlet fever
Schamberg disease...
Schistosomiasis
Schizencephaly
Schizophrenia
Schmitt Gillenwater Kelly...
Sciatica
Scimitar syndrome
Sciophobia
Scleroderma
Scrapie
Scurvy
Selachophobia
Selective mutism
Seminoma
Sensorineural hearing loss
Seplophobia
Sepsis
Septo-optic dysplasia
Serum sickness
Severe acute respiratory...
Severe combined...
Sezary syndrome
Sheehan syndrome
Shigellosis
Shingles
Shock
Short bowel syndrome
Short QT syndrome
Shprintzen syndrome
Shulman-Upshaw syndrome
Shwachman syndrome
Shwachman-Diamond syndrome
Shy-Drager syndrome
Sialidosis
Sickle-cell disease
Sickle-cell disease
Sickle-cell disease
Siderosis
Silicosis
Silver-Russell dwarfism
Sipple syndrome
Sirenomelia
Sjogren's syndrome
Sly syndrome
Smallpox
Smith-Magenis Syndrome
Sociophobia
Soft tissue sarcoma
Somniphobia
Sotos syndrome
Spasmodic dysphonia
Spasmodic torticollis
Spherocytosis
Sphingolipidosis
Spinal cord injury
Spinal muscular atrophy
Spinal shock
Spinal stenosis
Spinocerebellar ataxia
Splenic-flexure syndrome
Splenomegaly
Spondylitis
Spondyloepiphyseal...
Spondylometaphyseal...
Sporotrichosis
Squamous cell carcinoma
St. Anthony's fire
Stein-Leventhal syndrome
Stevens-Johnson syndrome
Stickler syndrome
Stiff man syndrome
Still's disease
Stomach cancer
Stomatitis
Strabismus
Strep throat
Strongyloidiasis
Strumpell-lorrain disease
Sturge-Weber syndrome
Subacute sclerosing...
Sudden infant death syndrome
Sugarman syndrome
Sweet syndrome
Swimmer's ear
Swyer syndrome
Sydenham's chorea
Syncope
Syndactyly
Syndrome X
Synovial osteochondromatosis
Synovial sarcoma
Synovitis
Syphilis
Syringomas
Syringomyelia
Systemic carnitine...
Systemic lupus erythematosus
Systemic mastocytosis
Systemic sclerosis
T
U
V
W
X
Y
Z
Medicines

Symptoms

SJS is characterized by fever, sore throat, and headache leading to the sudden development of circular mucocutaneous lesions (target lesions) that can cover the majority of the skin. These lesions begin as macules and can develop into papules, vesicles, blisters, or urticarial plaques. The most extreme cases are termed Toxic Epidermal Necrolysis Syndrome (TENS) or Lyell's Syndrome, in these cases the entire skin is affected.

Treatment

Treatment is initially similar to that of patients with thermal burns, and continued care can only be supportive (e.g. IV fluids) and symptomatic (e.g. analgesic mouth rinse for mouth ulcer); there is no specific drug treatment (2002). An ophthalmologist should be consulted if eyes are involved. Treatment with corticosteroids is controversial since it might aggravate the condition.

Cause

The cause of SJS is either infections (usually following viral infections such as herpes simplex virus, influenza, mumps, cat-scratch fever, histoplasmosis, Epstein-Barr virus, or similar), drug-induced (valdecoxib, penicillins, barbiturate, sulfas, phenytoin, lamotrigine, nevirapine), malignancy-related (carcinomas and lymphomas), or idiopathic (50% of the time)

Read more at Wikipedia.org


[List your site here Free!]


Kawasaki syndrome
From Gale Encyclopedia of Medicine, 4/6/01 by Rebecca J. Frey

Definition

Kawasaki syndrome is a potentially fatal inflammatory disease that affects several organ systems in the body, including the heart, circulatory system, mucous membranes, skin, and immune system. It occurs primarily in infants and children but has also been identified in adults as old as 34 years. Its cause is unknown.

Description

Kawasaki syndrome, also called mucocutaneous lymph node syndrome (MLNS), is an inflammatory disorder with potentially fatal complications affecting the heart and its larger arteries. Nearly twice as many males are affected as females. Although persons of Asian descent are affected more frequently than either black or white individuals, there does not appear to be a distinctive geographic pattern of occurrence. Eighty percent of cases involve children under the age of four. Although the disease usually appears in individuals, it sometimes affects several members of the same family and occasionally occurs in small epidemics.

Causes & symptoms

The specific cause of Kawasaki syndrome is unknown, although the disease resembles infectious illnesses in many ways. It has been suggested that Kawasaki syndrome represents an allergic reaction or other unusual response to certain types of infections. Some researchers think that the syndrome may be caused by the interaction of an immune cell, called the T cell, with certain poisons (toxins) secreted by bacteria.

Kawasaki syndrome has an abrupt onset, with fever as high as 104°F (40°C) and a rash that spreads over the patient's chest and genital area. The fever is followed by a characteristic peeling of the skin beginning at the fingertips and toenails. In addition to the body rash, the patient's lips become very red, with the tongue developing a "strawberry" appearance. The palms, soles, and mucous membranes that line the eyelids and cover the exposed portion of the eyeball (conjuntivae) become purplish-red and swollen. The lymph nodes in the patient's neck may also become swollen. These symptoms may last from two weeks to three months, with relapses in some patients.

In addition to the major symptoms, about 30% of patients develop joint pains or arthritis, usually in the large joints of the body. Others develop pneumonia, diarrhea, dry or cracked lips, jaundice, or an inflammation of the membranes covering the brain and spinal cord (meningitis). A few patients develop symptoms of inflammation in the liver (hepatitis), gallbladder, lungs, or tonsils.

About 20% of patients with Kawasaki syndrome develop complications of the cardiovascular system. These complications include inflammation of the heart tissue (myocarditis), disturbances in heartbeat rhythm (arrhythmias), and areas of blood vessel dilation (aneurysms) in the coronary arteries. Other patients may develop inflammation of an artery (arteritis) in their arms or legs. Complications of the heart or arteries begin to develop around the tenth day after the illness begins, when the fever and rash begin to subside. A few patients may develop gangrene, or the death of soft tissue, in their hands and feet. The specific causes of these complications are not yet known.

Diagnosis

Because Kawasaki syndrome is primarily a disease of infants and young children, the disease is most likely to be diagnosed by a pediatrician. The physician will first consider the possible involvement of other diseases that cause fever and skin rashes, including scarlet fever, measles, Rocky Mountain spotted fever, toxoplasmosis (a disease carried by cats), juvenile rheumatoid arthritis, and a blistering and inflammation of the skin caused by reactions to certain medications (Stevens-Johnson syndrome).

Once other diseases have been ruled out, the patient's symptoms will be compared with a set of diagnostic criteria. The patient must have a fever lasting five days or longer that does not respond to antibiotics, together with four of the following five symptoms:

  • Inflammation of the conjunctivae of both eyes with no discharge
  • At least one of the following changes in the mucous membranes of the mouth and throat: "strawberry" tongue; cracked lips; or swollen throat tissues
  • At least one of the following changes in the hands or feet: swelling caused by excess fluid in the tissues; peeling of the skin; or abnormal redness of the skin
  • A skin eruption or rash associated with fever (exanthem) on the patient's trunk
  • Swelling of the lymph nodes in the neck to a size greater than 1.5 cm.

Since the cause of Kawasaki syndrome is unknown, there are no laboratory tests that can confirm the diagnosis. The following test results, however, are associated with the disease:

  • Blood tests show a high white blood cell count, high platelet count, a high level of protein in the blood serum, and mild anemia.
  • Chest x ray may show enlargement of the heart (cardiomegaly).
  • Urine may show the presence of pus or an abnormally high level of protein.
  • An electrocardiogram may show changes in the heartbeat rhythm.

In addition to these tests, it is important to take a series of echocardiograms during the course of the illness because 20% of Kawasaki patients will develop coronary aneurysms or arteritis that will not appear during the first examination.

Treatment

Kawasaki syndrome is usually treated with a combination of aspirin, to control the patient's fever and skin inflammation, and high doses of intravenous immune globulin to reduce the possibility of coronary artery complications. Some patients with heart complications may be treated with drugs that reduce blood clotting or may receive corrective surgery.

Follow-up care includes two to three months of monitoring with chest x rays, electrocardiography, and echocardiography. Treatment with aspirin is often continued for several months.

Prognosis

Most patients with Kawasaki syndrome will recover completely, but about 1-2% will die as a result of blood clots forming in the coronary arteries or as a result of a heart attack. Deaths are sudden and unpredictable. Almost 95% of fatalities occur within six months of infection, but some have been reported as long as 10 years afterward. Long-term follow-up of patients with aneurysms indicates that about half show some healing of the aneurysm. The remaining half has a high risk of heart complications in later life.

Key Terms

Aneurysm
Dilation of an artery caused by thinning and weakening of the vessel wall.
Arrythmia
Abnormal heart rhythm.
Arteritis
Inflammation of an artery.
Cardiomegaly
An enlarged heart.
Conjunctivae
The mucous membranes that cover the exposed area of the eyeball and line the inner surface of the eyelids.
Exanthem
A skin eruption associated with a disease, usually one accompanied by fever as in Kawasaki syndrome.
Gangrene
The death of soft tissue in a part of the body, usually caused by obstructed circulation.
Hepatitis
Inflammation of the liver.
Meningitis
Inflammation of the membranes, called the meninges, covering the brain and spinal cord.
Mucocutaneous lymph node syndrome (MLNS)
Mucocutaneous lymph node syndrome, another name for Kawasaki syndrome. The name comes from the key symptoms of the disease, which involve the mucous membranes of the mouth and throat, the skin, and the lymph nodes.
Myocarditis
Inflammation of the heart muscle.
Stevens-Johnson syndrome
A severe inflammatory skin eruption that occurs as a result of an allergic reaction or respiratory infection.
T cell
A type of white blood cell that develops in the thymus gland and helps to regulate the immune system's response to infections or malignancy.

Further Reading

For Your Information

    Books

  • Abzug, Mark J. "Infectious Diseases: Bacterial, Spirochetal, Protozoal, Metazoal, & Mycotic." In Handbook of Pediatrics, edited by Gerald B. Merenstein, et al. Norwalk, CT: Appleton & Lange, 1994.
  • "Childhood Infections: Kawasaki Syndrome." In The Merck Manual of Diagnosis and Therapy, Vol. II, edited by Robert Berkow, et al. Rahway, NJ: Merck Research Laboratories, 1992.
  • Shaffer, Elizabeth M., et al. "Heart." In Handbook of Pediatrics, edited by Gerald B. Merenstein, et al. Norwalk, CT: Appleton & Lange, 1994.
  • Shandera, Wayne X., and Maria E. Carlini. "Infectious Diseases: Viral & Rickettsial." In Current Medical Diagnosis & Treatment 1998, edited by Lawrence M. Tierney Jr., et al. Stamford, CT: Appleton & Lange, 1997.
  • Wolfe, Robert R., et al. "Cardiovascular Diseases." In Current Pediatric Diagnosis & Treatment, edited by William W. Hay Jr., et al. Stamford, CT: Appleton & Lange, 1997.

Gale Encyclopedia of Medicine. Gale Research, 1999.

Return to Stevens-Johnson syndrome
Home Contact Resources Exchange Links ebay