Stevens-Johnson Syndrome

Stevens-Johnson syndrome
Last updated: Wednesday, August 17, 2011

BACKGROUND

Stevens-Johnson affection (SJS) is a blazon of allergic acknowledgment that usually occurs in acknowledgment to medications. It is a astringent blazon of erythema multiforme, which is a derma ache characterized by derma eruptions and blisters. SJS is a beneath astringent anatomy of accession ache alleged baneful epidermal necrolysis (TENS).

Pediatricians, A.M. Stevens and S.C. Johnson, apparent the ache in 1922 if they advised two adolescent macho patients with derma lesions, fever, affronted film and conjunctivitis (pinkeye).

SJS is a systemic ataxia that is potentially activity threatening, abnormally if larboard untreated. About 3-15% of patients with astringent SJS die. In astringent cases, the lesions can could could could could could could cause cogent scarring of the circuitous organs, which about leads to accident of activity of the agency systems. For instance, brake of the esophagus and of the respiratory amplitude can activity due to lesions and scaring.

SJS about involves abounding areas of the physique and all-encompassing bane formation. The lesions can extend to the close membranes and affect any organ, including the lungs, eyes, mouth, abdomen and intestines. The ache can could could could could could could cause astringent accident to the mouth, nose, eye, vagina, urethra, gastrointestinal amplitude and/or lower respiratory tract.

It is estimated that about one actor Americans are diagnosed with SJS anniversary year.

SJS is an immune-complex-mediated hypersensitivity ataxia that may be acquired by medications, viral infections and malignancies (cancers). In up to bisected of cases, no specific analysis has been identified.

The arrangement of macho to changeable accident is two to one. Since the causes of SJS are not able-bodied understood, it is cryptic why it is added accepted a allotment of males.

Currently, there is no analysis for the specific disease. Instead, analysis focuses on abating the symptoms. In general, the activity lasts about two to six weeks.

CAUSES

Drug-induced: Almost any medication, including over-the-counter medications, like ibuprofen (Motrin© or Advil©) may advance to SJS. Medications a lot of about associated with SJS cover penicillins and sulfa antibiotics.

Anticonvulsants (like phenytoin, carbamazepine, valproic acerbic and lamotrigine) and nonsteroidal ant-inflammatory drugs (NSAIDs) accept aswell acquired SJS.

In backward 2002, the U.S. Food and Biologic Administration (FDA) and the architect Pharmacia acclaimed that SJS had been appear in patients demography the cyclooxygenase-2 (COX-2) inhibitor valdecoxib.

There accept aswell been contempo letters of cocaine-induced SJS.

Herbs/supplements: Since abounding herbs or supplements accept biologic properties, they may aswell could could could could could could cause SJS in some patients. SJS has been appear with the use of ginseng-containing products, as able-bodied as belladonna and Ginkgo biloba.

Infection: Added than bisected of the patients with SJS address accepting had a contempo high respiratory amplitude infection afore the access of SJS. Viral diseases that are frequently associated with SJS cover canker canker virus (HSV), AIDS, coxsackie viral infections, influenza, hepatitis, mumps, mycoplasmal infection, lymphogranuloma venereum (LGV), rickettsial infections and variola (smallpox).

Tumor-related: Assorted cancers and lymphomas accept been associated with SJS. Tumor-related SJS is added accepted a allotment of adults than children.

Idiopathic: If the could could could could could could cause of SJS is unknown, it is advised idiopathic. About 25-50% of SJS cases are idiopathic.

ADVERSE EFFECT VS. ALLERGIC REACTION

Not all adverse reactions to drugs are allergies. In fact, beneath than 10 percent of adverse biologic reactions are allergic because a lot of biologic ancillary furnishings are not accompanying to the allowed system. Instead, the biologic anon affects assorted organs throughout the body.

For instance, a nasal decongestant constricts the capillaries in the adenoids in adjustment to abate nasal abscess and accessible the airway passages. However, a ancillary aftereffect is that it aswell constricts the capillaries in added regions of the body, which may advance to an added affection rate, headache, blackout or added claret pressure. Since a decongestant is not specific to a alone allotment of the physique or one alone action, abounding humans could acquaintance ancillary effects. Therefore, it is a non-allergic acknowledgment because the body’s allowed arrangement is not involved.

Other causes of adverse reactions cover interactions amid two or added drugs, balance or the body’s disability to breach the biologic down absolutely as a aftereffect of alarmist or branch damage.

Allergic reactions can be classified into four immunopathologic categories application assorted allocation systems. These classifications are based on the allowed system’s acknowledgment to the allergen, not on the severity of the reaction.

SJS is technically an immune-complex-mediated hypersensitivity (allergic) condition. Therefore, SJS is a blazon III allergic reaction. This class involves the accumulation of an antigen-antibody allowed complex, which deposits on claret barge walls and activates corpuscle apparatus accepted as complements. This causes a serum-sickness like syndrome, involving fever, swelling, derma adventurous and amplification of the lymph nodes in about three to eight hours. It may be acquired by a array of allergens, including penicillins, sulfonamides, intravenous (IV) adverse media and hydantoins.

SYMPTOMS

About 3-15% of patients with astringent SJS die. SJS is potentially activity aggressive because in astringent cases, the lesions can could could could could could could cause cogent scarring of the circuitous organs, which about leads to accident of activity of the agency systems.

The ache about starts with a all-embracing high respiratory amplitude infection. The accommodating may acquaintance flu-like symptoms, including, fever, abscessed throat, chill, cephalalgia and angst (generally activity of discomfort) for anywhere from one to 14 days. Some individuals will aswell acquaintance diarrhea and vomiting.

As the ache progresses, mucocutaneous (mucus film and the skin) lesions bound develop. Patients may acquaintance all-encompassing sloughing (shedding of the skin). Derma lesions may activity anywhere, but they are a lot of accepted on the palms, soles of the feet, aback of the easily and extremities. Mucosal captivation may cover erythema (reddening of the skin), edema (fluid in physique tissues), blistering, ulceration (open abscessed on the skin) and afterlife (dead skin). Clusters of outbreaks about endure about two to four weeks.

Lesions may become bullous and rupture, authoritative the derma accessible to accessory infection. The lesions usually do not itch.

Lesions in the aperture and/or close membranes may accomplish it difficult for the accommodating to eat or drink. Patients who acquaintance affection in the genitourinary amplitude may aswell acquaintance aching urination.

Other accepted affection of SJS include, assiduous fever, abscess of eyelids or red eyes, conjunctivitis (pinkeye), flu-like symptoms, tachycardia, low claret pressure, epistaxis (nosebleeds), seizures or coma.

SJS is a life-threatening reaction, if larboard untreated. Complications may cover abiding blindness, dry-eye syndrome, photophobia (sensitivity to light), lung damage, abiding adverse pulmonary ache (COPD), asthma, abiding accident of attach beds, scarring of the esophagus and added close membranes, arthritis and abiding fatigue syndrome. All-encompassing derma captivation may could could could could could could cause the accident of ample quantities of physique fluids, causing shock in accession to the accident of infection.

TREATMENT

If the activity is drug-induced, the afflicted alone should stop demography the behind medication immediately. No specific biologic analysis exists for SJS. Recovery may yield two to six weeks. Astringent cases may crave analysis in an accelerated affliction assemblage or bake unit, area the accommodating will accept intravenous fluids and comestible supplements.

Acetaminophen: Acetaminophen (Tylenol©) has been acclimated to abate agitation and ache associated with SJS.

Antibiotics: Antibiotics are assigned to amusement accessory infections that are about associated with SJS. Frequently assigned antibiotics cover penicillin, amoxicillin, erythromycin and cefadroxil.

Corticosteroids: The use of systemic corticosteroids is controversial. High doses taken aboriginal in the acknowledgment accept been effective. However, they ability access accident of accessory infections because of their immunosuppressive properties.

Immunosupressants: Immunosuppressants like cyclophosphamide and cyclosporine accept been acclimated to amusement allergic symptoms.

IVIG: Human intravenous immunoglobulin (IVIG) has been acclimated to amusement astringent cases of SJS. IVIG may advice anticipate accessory infections in patients adversity from SJS by accretion immunoglobulin levels in the bloodstream.

Moist compress: For balmy symptoms, a clammy abbreviate has been activated to derma lesions. Areas of bald derma should be covered with compresses of acrid or burow solution.

Oral rinse: Analgesic aperture bathe has been acclimated to amusement aperture ulcers associated with SJS.

Topical anesthetics: Topical anesthetics like lidocaine (Lidoderm©) accept been activated to the derma to abate affliction associated with lesions.

PREVENTION

Avoid acknowledgment to accepted antigens that accept acquired SJS in the past.

Consider cutting a medical active ID armlet or necklace.

SUPPORT FOR PATIENTS

The Steven Johnson Affection Foundation is an online ability for SJS patients and their families. The alignment aims to accommodate abutment services, advice about SJS treatments and therapies to both healthcare professionals and the public. The ambition of the alignment is to advance acquaintance about the ache so that a quick analysis can be fabricated and superior analysis can begin.

More advice can be begin at the Steven Johnson Affection Foundation website, www.sjsupport.org.

AUTHOR INFORMATION

This advice has been edited and peer-reviewed by contributors to the Natural Standard Research Collaboration (www.naturalstandard.com).

  • Hebert AA, Bogle MA. Intravenous immunoglobulin prophylaxis for alternate Stevens-Johnson syndrome. J Am Acad Dermatol 2004; 50(2): 286-288.

    View abstract

  • National Library of Medicine. www.nlm.nih.gov.
  • Natural Standard: The Authority on Integrative Medicine. www.naturalstandard.com. Copyright © 2008.
  • Stevens Johnson Affection Foundation. www.sjsupport.org.

Copyright © 2011 Natural Standard (www.naturalstandard.com)

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