Stevens Johnson Syndrome

Synonyms

• Dermatostomatitis, Stevens Johnson Type
• Ectodermosis Erosiva Pluriorificialis
• Erythema Multiforme Exudativum
• Erythema Polymorphe, Stevens Johnson Type
• Febrile Mucocutaneous Syndrome, Stevens Johnson Type
• Herpes Iris, Stevens-Johnson Type
• Johnson-Stevens Disease

Disorder Subdivisions

• None

General Discussion

Until recently the relationship of Stevens-Johnson syndrome to other severe blistering disorders was a matter of some debate. Now a consensus seems to be evolving that describes SJS as a rare disorder involving lesions of the mucous membranes along with small blisters on the reddish or purplish, flat, thickened patches of skin. As a result, SJS is now distinguished as a separate disorder from erythema multiforme major (EMM). SJS is now considered to be a less severe variant of toxic epidermal necrolysis (TEN).

SJS and TEN appear to be characterized by identical clinical signs and symptoms, identical treatment approach and identical prognosis. Patients diagnosed with TEN can present with symptoms ranging from 10% skin involvement and severe threat to the patient’s sight to a presentation involving 90% of the skin but only a modest threat to the patient’s sight.

SJS (and TEN) is an inflammatory disorder of the skin triggered by an allergic reaction to certain drugs including antibiotics, such as some sulfonamides, tetracycline, amoxicillin, and ampicillin. In some cases, nonsteroidal anti-inflammatory medications and anticonvulsants, such as Tegretol and phenobarbital have also been implicated. Over-the-counter medications may act as triggers as well. In some cases, it is also possible that the disorder may be traced to a reaction to an infection.

One report suggests that the term SJS be limited to cases in which less than 10% of the total body surface area is involved. The authors suggest that the term TEN be limited to cases in which 30% or more of the total body surface area is involved. The term SJS/TEN Overlap is used to describe patients in whom between 10-30% of the total body surface area involved.
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Resources

NIH/National Arthritis and Musculoskeletal and Skin Diseases Information Clearinghouse
1 AMS Circle
Bethesda, MD 20892-3675
USA
Tel: 3014954484
Fax: 3017186366
Tel: 8772264267
TDD: 3015652966
Email: NIAMSinfo@mail.nih.gov
Internet: http://www.niams.nih.gov/Health_Info

NIH/National Eye Institute
Building 31 Rm 6A32
31 Center Dr MSC 2510
Bethesda, MD 20892-2510
United States
Tel: 3014965248
Fax: 3014021065
Email: 2020@nei.nih.gov
Internet: http://www.nei.nih.gov/

NIH/National Institute of Allergy and Infectious Diseases
6610 Rockledge Drive
MSC 6612
Bethesda, MD 20892-6612
Tel: (301)496-5717
Fax: (301)402-3573
TDD: (800)877-8339
Internet: http://www.niaid.nih.gov/

Stevens Johnson Syndrome Foundation and Support Group
PO Box 350333
Westminster, CO 80035-0333
Tel: (303)635-1241
Fax: (303)635-1241
Email: sjsupport@aol.com
Internet: http://www.sjsupport.org

MUMS (Mothers United for Moral Support, Inc) National Parent-to-Parent Network
150 Custer Court
Green Bay, WI 54301-1243
USA
Tel: 9203365333
Fax: 9203390995
Tel: 8773365333
Email: mums@netnet.net
Internet: http://www.netnet.net/mums/

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc. ® (NORD). 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 orphan@rarediseases.org

Last Updated:  7/23/2007
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