Stevens Johnson Syndrome

Stevens Johnson Syndrome: Excerpt from The 5-Minute Pediatric Consult

Paul S. Matz, MD

Stevens Johnson Syndrome - BASICS

Stevens Johnson Syndrome - description

• Stevens-Johnson Syndrome (SJS) is a severe hypersensitivity reaction, characterized by diffuse bullous lesions, mucocutaneous involvement, and marked constitutional symptoms.
• SJS is often considered to lie on a spectrum between erythema multiforme minor (EM minor) on 1 extreme, and toxic epidermal necrolysis (TEN) on the other, but this classification is controversial. In this schema, SJS is defined as involving >2 mucous membranes and <10% of total body surface area, and TEN as involving >30%, with overlap in between.

Stevens Johnson Syndrome - epidemiology

Affects all ages, ethnicities, and both genders equally

Stevens Johnson Syndrome - incidence

Incidence is 2–3 cases per million people each year.

Stevens Johnson Syndrome - pathophysiology

• SJS is an acute hypersensitivity reaction, characterized by a cell-mediated immunologic reaction.
• A prodrome of 1–14 days is often seen, consisting of constitutional symptoms, including high fever, myalgias, and arthralgias.
• A rash often starts on the torso, face, and neck, but spreads to the entire body within a few days.
• The rash generally starts as macular lesions, which may resemble the “target lesions” seen in EM minor. The target lesions progress to raised, purpuric lesions and bullae. As these bullae coalesce, the epidermis may slough.
• Ulcers and bullae may form on all mucous membranes (oropharynx and nasopharynx, eyes, genitals; and the respiratory, urinary, and GI tracts).
• Ophthalmic involvement includes severe keratitis and conjunctivitis, which can lead to ulcerations, perforations, and scarring.
• The pathologic findings of SJS are an inflammatory infiltration of the epidermis progressing to full-thickness necrosis of the epidermis, which leads to sloughing.

Stevens Johnson Syndrome - etiology

SJS is an acute hypersensitivity reaction to the following:

• Medications: Classically, anticonvulsants, antibiotics (penicillins, sulfonamides, and erythromycin), NSAIDs, and allopurinol
• Infections: Specifically, herpes simplex virus (HSV) or Mycoplasma, but any viruses, bacteria, or fungi can be involved.
• Environmental factors: Foods, immunizations, and other chemical exposures
• Re-exposure to a causative agent can lead to more severe recurrences.

Stevens Johnson Syndrome - DIAGNOSIS

Stevens Johnson Syndrome - signs & symptoms

Stevens Johnson Syndrome - history

• Child has been on new medications in the last several weeks, specifically anticonvulsants or antibiotics
  • These medications are known to cause SJS, with the highest incidence in the 1st 8 weeks after initiation of therapy.
• Recent infections or chemical exposures
  • HSV, Mycoplasma, foods, and immunizations have all been associated with SJS.
• If child has had SJS previously, recurrences of SJS may occur and may be due to recurrent HSV infection.

Stevens Johnson Syndrome - physical exam

• Examine entire skin surface and all mucous membranes to determine extent of involvement. Also, look for signs of secondary infection.
• Nikolsky sign (sliding a finger across the affected skin causes sloughing of the epidermis) may be present.
• Evaluate hydration status.
• Evaluate respiratory, nutritional, GI, and urologic status.

Stevens Johnson Syndrome - tests

Stevens Johnson Syndrome - lab

There is no specific test for SJS. Laboratory tests should be used to support the diagnosis and to evaluate the extent of the illness.

• CBC: To screen for evidence of superinfection
• Electrolytes: To assess hydration status and renal function
• Liver function tests: To determine the presence of hepatitis
• Acute-phase reactants, including erythrocyte sedimentation rate: Often elevated
• Bacterial cultures (both blood and surface): May be used to assess for secondary infection
• Specific microbiologic tests: May be used to evaluate for HSV, Mycoplasma, or other causes of infection
• Chest x-ray and pulse oximetry: May be used to evaluate pulmonary status

Stevens Johnson Syndrome - differencial diagnosis

• Infections:
  • Varicella
  • Staphylococcal scalded skin syndrome
  • Measles (and other macular rashes)
• Rheumatologic:
  • Kawasaki disease
  • Henoch-Schonlein purpura
• Trauma:
  • Thermal or chemical burns
• Dermatologic:
  • Erythema multiforme minor
  • Fixed drug eruption
  • Pemphigus
• Other:
  • Graft-versus-host disease

Stevens Johnson Syndrome - TREATMENT

Stevens Johnson Syndrome - initial-stabilization

• Evaluate and address respiratory and circulatory problems (ABCs), if present.
• Evaluate and treat sepsis, if present.
• Children with significant pulmonary complications may require mechanical ventilation and should be managed in an ICU.
• Widespread skin involvement is an indication for burn unit admission, as these children will require intensive wound management by pediatric burn specialists.

Stevens Johnson Syndrome - general measures

• Therapy is supportive for most patients.
• If an offending drug is identified, it must be discontinued immediately, as this may reduce mortality.
• Fluid resuscitation and ongoing, aggressive fluid management are crucial.
• Many patients are unable to tolerate PO feedings because of ulcerations of the GI tract. Nasogastric or parenteral nutrition must be considered to provide supraphysiologic calorie requirements.
• Meticulous skin care, including topical care, débridement, and skin grafting
• Any eye involvement warrants urgent ophthalmologic evaluation. Ocular lubrication may be required.
• Close monitoring for bacterial superinfection, both superficial infections and sepsis
• Close monitoring of respiratory, renal, hepatic, or GI complications. Mechanical ventilation may be warranted in severe cases.
• Underlying medical conditions (e.g., epilepsy), may become exacerbated if chronic medications are discontinued.

Stevens Johnson Syndrome - medication

• Open skin lesions should be covered with an antibiotic ointment, such as bacitracin or chlorhexidine, and gauze (with or without petrolatum). Sulfa-containing antibiotics such as sulfadiazine may exacerbate SJS and should be avoided.
• Prophylactic antibiotics are not indicated. Treat with antibiotics only if diagnosed with secondary bacterial infections.
• Acyclovir may be used to treat HSV, if present. Consider using acyclovir empirically in cases of recurrent SJS.
• Systemic corticosteroids: Their use has been controversial for some time. Corticosteroids are of questionable efficacy for SJS and can increase the risk of infection.
  • Some authors have found benefit when used in high doses early in the course of disease.
    • Generally, corticosteroids should not be used for the majority of cases of SJS.
  • They may be considered in cases of recurrent SJS, when the disease is quickly identified, but for no >3 days, as their use raises the risk of superinfections.
• IV immunoglobulin: Some small studies have reported that doses of 0.5 mg/kg/d over 4 days may be effective at slowing the progression of SJS. IV immunoglobulin is proposed to work by blocking apoptosis induced by the inciting agent.
• There is no clear consensus on the use of IVIG in SJS. The available data does suggest a marked improvement when IVIG is used, but the small sample sizes make it difficult to generalize to all patients. The use of IVIG should be considered early in the course of SJS, weighing risks and benefits of treatment, as well as availability of IVIG.
• Plasmapheresis, cyclosporine, and cyclophosphamide have been investigated but have not clearly shown benefit.

Stevens Johnson Syndrome - FOLLOW UP

Stevens Johnson Syndrome - prognosis

• Average length of hospital stay is 3 weeks.
• Mortality is ~5%, with most children recovering with only minimal sequelae.
• Mortality increases with increasing skin involvement, approaching 20–25% for patients with TEN.
• Skin lesions generally heal without scarring, but pigment changes may occur.
• Loss of hair and nails is rare.
• Eyes are the major source of long-term morbidity, with chronic dryness, scarring, and rarely blindness.

Stevens Johnson Syndrome - complications

• Dehydration due to fluid loss from damaged skin
• Sepsis
• Bacterial superinfection
• Pneumonia, pneumonitis, pleural effusion, and respiratory failure (due to infection or sloughing of respiratory mucosa)
• GI bleeding, ulcers, diarrhea, or constipation (due to painful anal ulcerations)
• Hepatitis
• Urinary retention, nephritis, and renal failure
• Chronic eye dryness and scarring; rarely blindness

Stevens Johnson Syndrome - patient-monitoring

• Hydration and nutrition must be managed until the lesions are healed and the patient tolerates oral feedings.
• Meticulous skin care until all lesions are healed
• Long-term ophthalmologic follow-up is necessary if there has been any eye involvement.
• If a preceding exposure is identified, it must be avoided.
• Prophylactic acyclovir may be used in patients with recurrent SJS due to HSV.

Failure to recognize significant complications, especially infection and dehydration

Stevens Johnson Syndrome - bibliography

1. Forman R, Koren G, Shear NH. Erythema multiforme, Stevens-Johnson, and toxic epidermal necrolysis in children: A review of 10 years’ experience. Drug Saf. 2002;25:965–972.
2. French L, Trent J, Kerdel F. Use of intravenous immunoglobulin in toxic epidermal necrolysis and Stevens–Johnson syndrome: Our current understanding. Int Immunopharmacol. 2006;6:543–549.
3. Fritsch PO, Sidoroff A. Drug-induced Stevens-Johnson syndrome/toxic epidermal necrolysis. Am J Clin Dermatol. 2000;1:349–360.
4. Leaute-Labreze C, Lamireau T, Chawki D, et al. Diagnosis, classification, and management of erythema multiforme and Stevens-Johnson syndrome. Arch Dis Child. 2000;83:347–352.
5. Martinez AE, Atherton DJ. High-dose systemic corticosteroids can arrest recurrences of severe mucocutaneous erythema multiforme. Pediatr Dermatol. 2000;17:87–90.
6. Metry DW, Jung P, Levy ML. Use of intravenous immunoglobulin in children with Stevens-Johnson syndrome and toxic epidermal necrolysis: Seven cases and review of the literature. Pediatrics. 2003;112:1430–1436.
7. Mockenhaupt M, Messenheimer J, Tennis P, et al. Risk of Stevens–Johnson syndrome and toxic epidermal necrolysis in new users of antiepileptics. Neurology. 2005;64:1134–1138.
8. Prendiville J. Stevens-Johnson syndrome and toxic epidermal necrolysis. Adv Dermatol. 2002;16:151–173.

Stevens Johnson Syndrome - CODES

Stevens Johnson Syndrome - icd9

695.1 Stevens-Johnson syndrome

Stevens Johnson Syndrome - FAQ

• Q: When should corticosteroids be considered?
• A: In patients with recurrent SJS in whom the disease is quickly identified, corticosteroids may be of benefit if started early. This is the only instance when corticosteroids should be strongly considered. Corticosteroids started later in the illness are not helpful. They may also exacerbate underlying HSV infection.
• Q: Do all patients with SJS require an ophthalmologic evaluation?
• A: The presence of any ocular manifestation of SJS mandates ophthalmologic consultation, as eye involvement can progress and be quite severe. Children with no signs of eye involvement (redness, dryness, pain) can be managed without an ophthalmologic evaluation.
• Q: How should seizures be managed in patients with epilepsy who develop SJS from their antiepileptic medication?
• A: It is vital that the causative medication be discontinued immediately. Seizures should be managed with benzodiazepines until the SJS begins to resolve. At that point, neurologic consultation should be obtained to assist in choosing an antiepileptic medication, as the initial, causative medication cannot be used again.

Book Source Details

• Book Title: The 5-Minute Pediatric Consult
• Author(s): M. William Schwartz MD; et al.
• Year of Publication: 2008
• Copyright Details: The 5-Minute Pediatric Consult, Copyright © 2008 Lippincott Williams & Wilkins.

More About Stevens-Johnson Syndrome

• Back to disease: Stevens-Johnson Syndrome: Introduction
• Next Book Extract About Stevens-Johnson Syndrome: Surveys relating to Stevens-Johnson Syndrome
• More Book Extracts: All Online Books for Stevens-Johnson Syndrome

More Medical Textbooks Online about Stevens-Johnson Syndrome

Review other book chapters online related to Stevens-Johnson Syndrome:

Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.

More About This Book: Title: The 5-Minute Pediatric Consult Authors: M. William Schwartz MD; et al. Publisher: Lippincott Williams & Wilkins Copyright: 2008 ISBN: 0-7817-7577-9

 » Next page: Surveys relating to Stevens-Johnson Syndrome

Rate This Website

What do you think about the features of this website? Take our user survey and have your say:

Website User Survey

Medical Tools & Articles:

Next articles:

• Surveys relating to Stevens-Johnson Syndrome
• Ask Question about Stevens-Johnson Syndrome

Tools & Services:

• Bookmark this page
• Symptom Search
• Symptom Checker
• Medical Dictionary
• Give your feedback

Medical Articles:

• Disease & Treatments Search
• Misdiagnosis Center
• Full list of interesting articles

Forums & Message Boards

• Ask or answer a question at the Boards:
• I cannot get a diagnosis. Please help.
• Tell us your medical story.
• Share your misdiagnosis story.
• What is the best treatment for my condition?
• See all the Boards.

Enter your search terms Submit search form
Search The Web Search wrongdiagnosis.com

Common Health Mistakes

Symptom
Checker

Search Specialists by State and City