Stevens Johnson Syndrome

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Important

It is possible that the main title of the report Stevens Johnson Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

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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Last updated:	July 23, 2007