$Unique_ID{BRK03947} $Pretitle{} $Title{Lyelles Syndrome} $Subject{Lyelles Syndrome TEN Scalded Skin Syndrome Staphylococcal Scalded Skin Syndrome Dermatitis Exfoliativa Ritter-Lyell Syndrome Ritter Disease Lyell Syndrome Acute Toxic Epidermolysis Bullosa Stevens-Johnson Syndrome} $Volume{} $Log{} Copyright (C) 1987, 1989 National Organization for Rare Disorders, Inc. 414: Lyelles Syndrome ** IMPORTANT ** It is possible the main title of the article (Lyelles Syndrome) is not the name you expected. Please check the SYNONYMS listing on the next page to find alternate names, disorder subdivisions, and related disorders covered by this article. Synonyms TEN Scalded Skin Syndrome Staphylococcal Scalded Skin Syndrome Dermatitis Exfoliativa Ritter-Lyell Syndrome Ritter Disease Lyell Syndrome Acute Toxic Epidermolysis Information on the following diseases can be found in the Related Disorders section of this report: Epidermolysis Bullosa Stevens-Johnson Syndrome 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. Lyelles Syndrome is a serious skin disorder characterized by severe redness, blisters and peeling. Onset can begin during any stage of life. The infantile form may follow an infection. In adults the disorder is often caused by a drug reaction. Treatment of cases caused by infection may differ from those caused by drug reactions. Symptoms Symptoms of infantile or childhood onset Lyelles Syndrome may have a preliminary stage marked by fever, sore throat, eye inflammation (conjunctivitis), nasal discharge, vomiting, diarrhea and/or back pain. Development of crusted lesions around the nose or ear can be followed within twenty-four hours by intense redness in the same area. Skin redness may spread with tenderness, itching and separation of skin layers possibly developing within thirty-six to forty-eight hours. Skin may peel away in large sheets when touched. Blisters of various sizes may form along with peeling. The severity of peeling skin is often related to the degree of fever, severe discomfort, and loss of appetite. With healing, peeled areas may become dried and yellowish crusts may appear in affected areas. Large, easily broken blisters may be the first apparent symptom of adult onset Lyelles Syndrome. These blisters may be associated with other skin conditions such as Stevens-Johnson Syndrome (Erythema Multiforme Bullosum). Large sheets of skin can peel due to slight injury or simply touching an object and the mucous membranes may also be involved. Lyelles Syndrome usually progresses rapidly and within a few days may become severe. Fluid may be lost after extensive peeling occurs possibly leading to dehydration like that which occurs in burn victims. Scars resembling those of burns may develop when the skin begins to heal. Causes Lyelles Syndrome is often caused by bacterial (staphylococcal) infections when infants or children are affected. A common staphylococcal skin infection with blisters and crusting called Impetigo may also precede this disorder. Lyelles Syndrome can occur sporadically or in epidemic proportions in nurseries. An allergic reaction to a drug often causes symptoms to develop during adulthood. Very rarely, some childhood cases may be linked to a drug reaction. Affected Population Lyelles Syndrome affects males and females in equal numbers. Related Disorders Symptoms of the following disorders can be similar to those of Lyelles Syndrome. Comparisons may be useful for a differential diagnosis: Epidermolysis Bullosa (EB) is the name of a group of rare, hereditary skin disorders in which blisters develop on many areas of the body. Severe forms of the disease may include involvement of the mucous membranes, internal digestive tract and other organs. Infection must be guarded against and often scars cannot be avoided. (For more information on this disorder, choose "EB" as your search term in the Rare Disease Database). Stevens-Johnson Syndrome is another blistering skin disorder usually affecting children and young adults. It is marked by severe reddening and blisters on the skin and mucous membranes. A preliminary stage may last from one to two weeks consisting of fever, discomfort, coughing linked to an acute allergic reaction, sore throat, chest pain, vomiting, diarrhea, and joint or muscle pain. Lesions may then appear in the mouth and throat as well as on reproductive organs and the anal area. Progressive eye inflammation can lead to diminished vision. Crusting with bleeding may develop on the lips. Symptoms may heal within three to six weeks, but can recur at any time. The cause of this disorder is not known, although more severe cases are possibly associated with collagen disease, malignancy, contact dermatitis or drug reactions. Stevens-Johnson usually appears in the fall or spring, for reasons not yet understood by scientists. (For more inforation on this disorder, choose "Stevens-Johnson" as your search term in the Rare Disease Database.) Therapies: Standard Early treatment of infantile or childhood onset Lyelles Syndrome is recommended because of the rapid progression of symptoms. Antibiotic (e.g., penicillin) drug therapy may be helpful for treatment of infection. Fluid and electrolyte balance may need correction. Children should be watched carefully to prevent them from touching peeled or blistered areas which could possibly worsen the condition. Hospitalization and/or isolation may be required to assure a sterile environment. Healing may rapidly follow appropriate treatment. Treatment of adult onset Lyelles Syndrome is usually similar to therapy for severe burns. Contact with peeled skin surface should be minimal. Hospitalization with isolation in a sterile environment to minimize infection may be necessary. Severe loss of fluid and electrolytes may require replacement on an ongoing basis to prevent dehydration. If the disorder is caused by a drug reaction, systemic corticosteroids may be able to control the reaction but do not seem to improve skin symptoms. Blood poisoning and lung infections should be anticipated and treated promptly if they occur. Other therapy is symptomatic and supportive. Therapies: Investigational Experimental plasmapheresis may be of benefit in cases of Lyelles Syndrome caused by a severe reaction to drugs. This procedure is a method for removing unwanted substances (toxins, metabolic substances and plasma parts) from the blood. Blood is removed from the patient and blood cells are separated from plasma. The patient's plasma is then replaced with other human plasma and the blood is retransfused into the patient. This therapy is still under investigation to analyze possible side effects and effectiveness. More research is needed before plasmapheresis can be recommended for use in all but the most severe cases of Lyelles Syndrome. Research is underway in the areas of new wound healing drugs, antibiotics and the inhibition of blister formation at dermatology research centers listed in the Resources section of this report. This disease entry is based upon medical information available through October 1988. 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 on this disorder. Resources For more information on Lyelles Syndrome, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 The National Arthritis and Musculoskeletal and Skin Diseases Information Clearinghouse Box AMS Bethesda, MD 20892 (301) 495-4484 Dystrophic Epidermolysis Bullosa Research Association of America (DEBRA) 141 Fifth Ave., Suite 7-South New York, NY 10010 (212) 995-2220 D.E.B.R.A. 7 Sandhurst Lodge Wokingham Road Crowthorne Berkshire RG11 7QD England Tel: 0344 771961 CLINICAL FACILITIES University of Washington School of Medicine Department of Dermatology St. Louis, MO 63110 (314) 362-5000 Rockefeller University Department of Investigative Dermatology 1230 York Avenue New York, NY 10021 (212) 570-8000 Children's Hospital Department of Pediatric Dermatology Dermatology Clinic 34th and Civic Center Blvd. Philadelphia, PA 19104 (215) 596-9100 University of Pennsylvania Dermatology Clinic 34th and Spruce Street Philadelphia, PA 19104 (215) 622-2737 References IMPROVED BURN CENTER SURVIVAL OF PATIENTS WITH TOXIC EPIDERMAL NECROLYSIS MANAGED WITHOUT CORTICOSTEROIDS: P.H. Halebian, et al.; Ann Surg (November 1986, issue 204(5)). Pp. 503-512. PLASMAPHERESIS IN SEVERE DRUG-INDUCED TOXIC EPIDERMAL NECROLYSIS: D. Kamanabroo, et al.; Arch Dermatol (December 1985, issue 121(12)). Pp. 1548- 1549.