$Unique_ID{BRK04236} $Pretitle{} $Title{Stevens-Johnson Syndrome} $Subject{Stevens-Johnson Syndrome Erythema Multiforme Exudativum Ectodermosis Erosiva Pluriorificialis Johnson-Stevens disease Febrile Mucocutaneous Syndrome Herpes Iris Dermatostomatitis Erythema Polymorphe } $Volume{} $Log{} Copyright (C) 1986, 1989 National Organization for Rare Disorders, Inc. 295: Stevens-Johnson Syndrome ** IMPORTANT ** It is possible the main title of the article (Stevens-Johnson Syndrome) is not the name you expected. Please check the SYNONYMS listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Erythema Multiforme Exudativum Ectodermosis Erosiva Pluriorificialis Johnson-Stevens disease Febrile Mucocutaneous Syndrome Herpes Iris Dermatostomatitis Erythema Polymorphe General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Stevens-Johnson syndrome is a severe form of Erythema Multiforme characterized by blistery lesions on the mucous membranes of the mouth, throat, anogenital region, eyelids and corneal lining (conjunctiva). Symptoms Stevens-Johnson syndrome is characterized by blistery eruptions on the mucous membranes of the mouth, throat, anogenital region, intestinal tract and eyelid lining (conjunctiva). Typical Erythema Multiforme lesions may or may not be present elsewhere on the skin. The patient with Stevens-Johnson syndrome may be unable to eat or close his/her mouth properly. Drooling may occur as a result. Eyes may become painful and inflammation of the eyelids (conjunctivitis) with swelling and pus may cause great discomfort. Untreated, the conjunctival lesions may leave residual corneal scarring, which sometimes may cause blindness. A patchy pneumonia is often present in the lungs. There may also be symptoms of joint inflammation and general fatigue. Causes 1. In about 50% of cases no cause can be found for Stevens-Johnson syndrome. 2. In children and young adults, infections caused by herpes simplex virus are the most common cause. Additionally, coxsackie- and echoviruses, Mycoplasma pneumonia, psittacosis, histioplasmosis, and Vaccinia, Bacillus Calmette-Guerin, and poliomyelitis vaccines have been identified as causes of Stevens-Johnson syndrome. 3. In other cases, x-ray therapy or drugs can sometimes cause Stevens- Johnson syndrome in susceptible people. Penicillin, sulfonamides, and barbiturates are the most common drug causes. The mechanisms by which infectious agents or drugs can cause the condition in some people is unknown, but it appears to be an unpredictable allergic reaction to these substances. Affected Population People of all ages and both sexes can be affected by Stevens-Johnson syndrome. Related Disorders Allergic Stomatitis is an inflammation of the mouth characterized by an intense shiny redness of the mucous membrane in the mouth, accompanied by slight swelling, itching, dryness or burning sensation. This disorder may be due to sensitivity to foods or lipstick. Herpetic Stomatitis is an inflammation of the mouth caused by the herpes simplex virus and characterized by itching followed by the appearance of small tense blisters on a red base. Reiter's syndrome is a combination of arthritis, inflammation of the urethra (urethritis), and conjunctivitis. It is similar to Stevens-Johnson syndrome, but distinguished by a definite burning sensation during urination and the frequent finding of hard, crusted lesions on the feet, hands, and occasionally elsewhere. Mikulicz syndrome (aphthous stomatitis) is limited to the lips and mouth. It is a disorder without fever, characterized by recurrent red spots that may ulcerate, and last about 2 to 3 weeks at each occurrence. Behcet's syndrome is a triad of symptoms including lesions of the mouth, ulcerations of the genital mucous membranes, and inflammation of the eyes. The ulcerations are small, but deep and chronic. Stevens-Johnson syndrome is the most severe form of Erythema Multiforme. For more information on the preceding disorders, choose "Reiter," "Mikulicz," "Behcet," and "Erythema Multiforme" as your search terms in the Rare Disease Database. Therapies: Standard When a cause for Stevens-Johnson syndrome can be found, it should be treated, eliminated or avoided (e.g., drugs or other substances to which the patient is allergic). Local treatment depends on the type of lesion. Antibiotic therapy and treatment with glucocorticoids may relieve the symptoms and shorten the course of the disease. Glucocorticoids usually are not used if an infection is present. Infections of the lips and mouth may require special care. Intense oral hygiene is necessary. A mouthwash of sodium bicarbonate solution in warm water can be soothing and cleansing. Rinsing after each meal with elixir of dexamethasone can relieve discomfort and promote healing of nonviral oral lesions. Another mouthwash that can be helpful is a solution of betnesol, zephiran HCl, peppermint oil, lidocaine HCl and tragacanth in water. Systemic corticosteroids have often been used in Stevens-Johnson syndrome, sometimes with apparent benefit. Intensive systemic antibiotics, fluids and electrolytes may be lifesaving in patients with extensive mucous membrane lesions. Ophthalmic consultation is required when the eyes are involved. Precautions must be taken to avoid permanent eye damage. Therapies: Investigational This disease entry is based upon medical information available through March 1987. 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. Resources For more information on Stevens-Johnson Syndrome, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 NIH/National Institute of Allergy and Infectious Diseases 9000 Rockville Pike Bethesda, MD 20892 (301) 496-5717 The National Arthritis and Musculoskeletal and Skin Diseases Information Clearinghouse Box AMS Bethesda, MD 20892 (301) 495-4484 NIH/National Eye Institute 9000 Rockville Pike Bethesda, MD 20892 (301) 496-5248 References ERYTHEMA MULTIFORME: W. Stewart, et. al., eds; In: Dermatology, Diagnosis and Treatment of Cutaneous Disorders, 3rd ed. Mosby, 1984.