Sweet Syndrome

Sweet Syndrome

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Sweet 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

  • Febrile Neutrophilic Dermatosis, Acute

Disorder Subdivisions

  • None

General Discussion

Sweet syndrome is a rare skin disorder characterized by fever, inflammation of the joints (arthritis), and the sudden onset of a rash. The rash consists of bluish-red, tender papules that usually occur on the arms, legs, face or neck, most often on one side of the body (asymmetric). In approximately 80 percent of cases, Sweet syndrome occurs by itself for no known reason (idiopathic). In 10 to 20 percent of cases, the disorder is associated with an underlying malignancy, usually a hematologic malignancy such as certain types of leukemia. The exact cause of Sweet syndrome is unknown.

Symptoms

The major symptom of Sweet syndrome is the sudden onset of bluish-red, tender papules on the arms, legs, face or neck. These skin lesions may also occur on the thighs and trunk. The lesions are sometimes up to an inch in diameter, flat or slightly elevated, irregularly-shaped, and inflamed. The lesions slowly grow larger and last for at least two weeks. The symptoms of Sweet syndrome often subside but tend to recur chronically.



Individuals with Sweet syndrome also experience fever, a general feeling of ill health (malaise), inflammation and pain of the joints (arthritis and arthralgia). In addition, affected individuals may experience inflammation of the delicate membrane that lines the eyes (conjunctivitis). In some cases, individuals with Sweet syndrome may have lesions affecting the mucous membranes of the mouth (oral mucosa).



In most cases (approximately 80 percent), Sweet syndrome occurs without any underlying disorder. In these cases, the onset of Sweet syndrome usually follows an infection of the upper respiratory tract or gastrointestinal system.



In the remaining 20 percent of cases, Sweet syndrome is associated with a malignancy, most often a malignancy that affects the blood (hematologic malignancies), such as certain types of leukemia and, rarely, cancers of the genitourinary and gastrointestinal tracts.



In some cases, Sweet syndrome may also be associated with autoimmune and inflammatory disorders such as Behcet's disease, Sjogren's syndrome, ulcerative colitis or Crohn's disease. (For more information on these disorders, see the Related Disorders section of this report.)

Causes

The exact cause of Sweet syndrome is not known. Some researchers speculate that Sweet syndrome occurs as an allergic reaction to an unknown agent, as a response to an infectious disease, or as a result of contact with certain chemicals (chemical irritants).



According to the medical literature, some cases of Sweet syndrome have occurred after the use of certain drugs, especially granulocyte-monocyte colony stimulating factor.



Patients often have an upper respiratory infection, tonsillitis, or influenza-like illness that precedes their skin lesions by one to three weeks.



Approximately 10 to 20 percent of cases have an associated malignancy. The associated malignancies include leukemias and lymphomas, as well as breast, genitourinary, and gastrointestinal tumors. Sweet syndrome has also been associated with inflammatory states, such as inflammatory bowel disease, toxoplasmosis, salmonellosis, tuberculosis, and vaginal infections. Pregnancy-associated Sweet syndrome typically presents in the first or second trimester. There does not appear to be any fetal risk, and the syndrome may recur with subsequent pregnancies.

Affected Populations

Sweet syndrome usually affects females in the fifth and sixth decades of life. However, cases of Sweet syndrome affecting males, children, and infants have been reported. More than 425 cases have been reported in the medical literature.

Standard Therapies

Diagnosis

A diagnosis of Sweet syndrome is made based upon a thorough clinical evaluation, a detailed patient history, identification of classic symptoms, and a variety of specialized tests. In many cases, surgical removal (biopsy) and microscopic examination of small samples of skin tissue may reveal abnormally high levels of a certain white blood cell (neutrophils) in the blood (neutrophilia).



Treatment

The treatment of Sweet syndrome is directed toward the specific symptoms that are apparent in each individual. In some cases, Sweet syndrome may resolve itself with no treatment. In most cases, treatment with low doses of corticosteroids such as methylprednisolone or prednisone has proven effective in eliminating symptoms. However, Sweet syndrome often recurs periodically despite therapy.



Individuals with Sweet syndrome should receive a thorough clinical examination to detect any possible underlying malignancy or disorder that may be associated with Sweet syndrome, including a complete hematologic evaulation.



Other treatment is symptomatic and supportive.

Investigational Therapies

Alternate drug therapies have been used to treat individuals with Sweet syndrome including indomethacin, potassium iodine, colchicines, cyclosporin, and dapsone. More studies are needed to determine the long-term safety and effectiveness of these drugs for the treatment of Sweet syndrome.



Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.



For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com

References

Odom RB, et al. Sweet's syndrome (acute febrile neutrophilic dermatosis) in Andrews' Diseases of The Skin. 9th ed. Philadelphia, PA: W.B. Saunders Company; 2000.155-57.



Bennett JC, Plum F, eds. Cecil Textbook of Medicine. 20th ed. Philadelphia, PA: W.B. Saunders Co; 1996:1019.



Champion RH, et al., eds. Textbook of Dermatology. 5th ed. Cambridge, MA: Blackwell Scientific Publications; 1992:1928-9.



Magalini SI, et al., eds. Dictionary of Medical Syndromes. 4th ed. New York, NY: Lippincott-Raven Publishers; 1997:776.



Inomata N, et al. Sweet's syndrome with gastric cancer. J Am Acad Dermatol. 1999;41:1033-4.



Petermann A, et al. Sweet's syndrome in a patient with acute Crohn's colitis and longstanding ankylosing spondylitis. Clin Exp Rheumatol. 1999;17:607-10.



Lopez de Maturana D, et al. Sweet syndrome associated with articular and renal involvement. Rev Med Chil. 1999;127:463-7.



Levi I, et al. Acute neutrophilic dermatosis induced by all-trans-retinoic acid treatment for acute promyelocytic leukemia. Leuk Lymphoma. 1999;34:401-4.



Arbetter KR, et al. Case of granulocyte colony-stimulating factor-induced Sweet's syndrome. Am J Hematol. 1999;61:126-9.



Tuerlinchkx D, et al. Sweet's syndrome with arthritis in an 8-month-old boy. J Rheumatol. 1999;26:440-2.



Hasegawa M, et al. Sweet's syndrome associated with granulocyte colony-stimulating factor. Eur J Dermatol. 1998;8:503-5.



Thurnheer R, et al. Bronchial manifestation of acute febrile neutrophilic dermatosis (Sweet's syndrome). Eur Respir J. 1998;11:978-80.



Salmon P, et al. A continuum of neutrophilic disease occurring in a patient with ulcerative colitis. Australas J Dermatol. 1998;39:116-8.



Ginarte M, et al. Sweet's syndrome: a study of 16 cases. Med Clin (Barc). 1997;109:588-91



Bourke JF, et al. Sweet's syndrome and malignancy in the U.K. Br J Dermatol. 1997;137:609-13.



Travis S, et al. Sweet's syndrome: an unusual cutaneous feature of Crohn's disease or ulcerative colitis. Eur J Gastroenterol Hepatol. 1997;9:715-20.



Mendoza H, et al. Sweet's syndrome. Presentation of six cases and review of the literature. An Med Interna. 1997;14:244-6.



Jeanfils S, et al. Indomethacin treatment of eighteen patients with Sweet's syndrome. J Am Acad Dermatol. 1997;36:436-9.



Lear JT, et al. Neutrophilic dermatoses: pyoderma gangrenosum and Sweet's syndrome. Postgrad Med J. 1997;73:65-8.



Silverman MA, et al. A case presentation of Sweet's syndrome and discussion of life-threatening dermatoses. Am J Emerg Med. 1996;14:165-9.



Jain KK, et al. Sweet's syndrome associated with granulocyte colony-stimulating factor. Cutis. 1996;57:107-10.



Su WP, et al. Sweet syndrome: acute febrile neutrophilic dermatosis. Semin Dermatol. 1995;14:173-8.



Fett DL, et al. Sweet's syndrome: systemic signs and symptoms and associated disorders. Mayo Clin Proc. 1995;70:234-40.



Reuss-Borst MA, et al. The possible role of G-CSF in the pathogenesis of Sweet's syndrome. Leuk Lymphoma. 1994;15:261-4.



von den Driesch P, et al. Sweet's syndrome-therapy with cyclosporin. Clin Exp Dermatol. 1994;19:274-7.



Boatman BW, et al. Sweet's syndrome in children. South Med J. 1994;87:193-6.

Resources

NIH/National Institute of Arthritis and Musculoskeletal and Skin Diseases

Information Clearinghouse

One AMS Circle

Bethesda, MD 20892-3675

USA

Tel: (301)495-4484

Fax: (301)718-6366

Tel: (877)226-4267

TDD: (301)565-2966

Email: NIAMSinfo@mail.nih.gov

Internet: http://www.niams.nih.gov/



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



Madisons Foundation

PO Box 241956

Los Angeles, CA 90024

Tel: (310)264-0826

Fax: (310)264-4766

Email: getinfo@madisonsfoundation.org

Internet: http://www.madisonsfoundation.org



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, 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.

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