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- $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.
-
-