$Unique_ID{BRK04246} $Pretitle{} $Title{Sweet Syndrome} $Subject{Sweet Syndrome Febrile Neutrophilic Dermatosis, Acute Erythema Multiforme Leiner Disease Ritter Disease (Dermatitis Exfoliativa Neonatorum) Erythema Elevatum Diutinum} $Volume{} $Log{} Copyright (C) 1987, 1989 National Organization for Rare Disorders, Inc. 424: Sweet Syndrome ** IMPORTANT ** It is possible the main title of the article (Sweet 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 Febrile Neutrophilic Dermatosis, Acute Information on the following disorders can be found in the Related Disorders section of this report: Erythema Elevatum Diutinum Erythema Multiforme Leiner Disease Ritter Disease (Dermatitis Exfoliativa Neonatorum) 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. Sweet Syndrome is a rare skin disorder characterized by painful red eruptions usually on the arms, face, neck, and legs. The exact cause of this disorder is not known, although it may be associated with an injury to the skin. Symptoms Major symptoms of Sweet Syndrome are tender or painful skin eruptions and generalized discomfort (malaise). Skin lesions usually occur on the arms, but also on the face, neck, legs, and occasionally the thighs and trunk. The lesions may be up to an inch in diameter. They are usually bluish-red, irregular, flat or raised, sharply outlined, circular, and/or hardened, with a rounded edge. Blisters or bacteria-free pimples usually cover the plaques. Scarring is usually absent. Fever may occur. Protein in the urine is sometimes present. Remission may occur after a few weeks, but recurrences are possible. Tests usually reveal infiltration of white blood cells called neutrophils in the skin. On rare occasions, the female genital tract (vagina and uterus) may be involved. Causes The exact cause of Sweet Syndrome is not known. Possibly it is an allergic reaction to an unknown agent. The disorder may be associated with an injury to the skin such as vaccination or a scrape. An upper respiratory or skin infection may also precipitate Sweet Syndrome. Affected Population Sweet Syndrome affects mainly middle aged females, but it may also affect men, infants and children in rare cases. It is a rare disorder. Related Disorders Symptoms of the following disorders can be similar to those of Sweet Syndrome. Comparisons may be useful for a differential diagnosis: Erythema Multiforme is an inflammatory skin disorder characterized by symmetric, red, blistery lesions appearing on the skin of the hands and feet. Mucous membranes and skin of the eyelids may also be affected. (For more information on this disorder, choose "Erythema Multiforme" as your search term in the Rare Disease Database.) Erythema Elevatum Diutinum is possibly a variant of Erythema Multiforme. It is a rare chronic skin disorder usually occurring in adults between 30 and 60 years of age. This disorder may be associated with recurrent polyarthritis and is characterized by symmetric nodules and plaques near the joints. These lesions commonly appear on the back of the hands and feet. The size of the lesions may vary over the course of a day. Leiner Disease is a skin disorder which usually occurs during the first two months of life. A reddish patch of thickened skin appears first on the buttocks and spreads to other parts of the infant's body. Scaling and peeling may occur as well as anemia, itching and diarrhea. The redness and scaliness usually decrease after a few weeks with treatment. (For more information on this disorder, choose "Leiner" as your search term in the Rare Disease Database.) Ritter Disease (Dermatitis Exfoliativa Neonatorum) is a skin disorder affecting infants which is usually caused by a bacterial infection. Reddened skin may peel leaving raw areas which heal in dry crusty yellow patches. This disorder may follow upper respiratory infections, impetigo, or other improperly treated skin infections. Therapies: Standard Sweet Syndrome may go into spontaneous remission after a few weeks even without treatment. Systemic steroid drugs such as prednisone may produce dramatic improvement. Therapies: Investigational The investigational drug dapsone has been used experimentally to treat Sweet Syndrome. However, more research is needed before this drug can be prescribed for general use. This disease entry is based upon medical information available through October 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 Sweet 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 References ACUTE FEBRILE NEUTROPHILIC DERMATOSIS. SWEET'S SYNDROME: M.A. Bechtel, et al.; Archives Dermatol (October 1981: issue 117,10). Pp. 664-666. SWEET'S SYNDROME: HISTOLOGICAL AND IMMUNOHISTOCHEMICAL STUDY OF 15 CASES: J.J. Going, et al.; Journal Clin Pathol (February 1987: issue 40,2). Pp. 175-179. ACUTE FEBRILE NEUTROPHILIC DERMATOSIS: SWEET'S SYNDROME: H. Chmel, et al.; South Med Journal (November 1978: issue 71,11). Pp. 1350-1352.