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Sweet Syndrome
Sunday, 16 November 2003
Thursday, 05 February 2004
Acute Febrile Neutrophilic Dermatosis

What

Sweet syndrome, also known as acute febrile neutrophilic dermatosis, is a reactive inflammatory disorder that includes fever, an increase in the body’s white blood cells and tender, reddish to purple skin lesions that can occur on the head, neck or trunk.

Who

Sweet syndrome, also known as acute febrile neutrophilic dermatosis, is a reactive inflammatory disorder that includes fever, an increase in the body’s white blood cells and tender, reddish to purple skin lesions that can occur on the head, neck or trunk.

Signs and Symptoms

The first symptom is usually the skin findings. Each group of lesions usually appears with a fever and can last from a few days to a few years (rare). Lesions are painful and can appear reddish-blue or violet, and show up as small to medium sized nodules that occasionally have pus within them. These lesions are found in an asymmetric (one-sided) distribution and can be found on the face, neck, trunk or extremities. Interestingly, the skin can demonstrate pathergy, which means that the lesions can occur in areas following minor damage to the skin such as a bite or scratch. Headache, muscle pain, joint pain, mouth sores and reddish eyes also commonly affect individuals with Sweet syndrome. The other organ systems affected by the disease include the lungs (increased cough or an abnormal chest x-ray), kidneys (protein or blood in the urine), or the nervous system (signs of inflammation in the fluid around the brain and spinal cord). A few children with Sweet syndrome also have been reported to get areas of inflammation within the bones that appear like infections, but have no bacteria within them.

Possible Causes

The cause of Sweet syndrome is not clearly understood at this time. It is thought that the body has an excessive reaction to some stimulus and releases extra white blood cells and other inflammatory chemicals to help the body in this time of stress. It is these chemicals, or cytokines, that cause the various signs and symptoms of Sweet syndrome. In about 60-70% of the cases, the cause is unknown. Sweet syndrome can also be associated with another disease. In adults, it has been shown to occur with certain malignancies like leukemia, lymphoma, osteosarcoma, oral cancer, ovarian cancer, prostate cancer, or testicular cancer. Fifteen percent of the time Sweet syndrome has been shown to be associated with an inflammatory disease like crohn disease, Behcet syndrome, rheumatoid arthritis or ulcerative colitis. Other minor causes that have been identified are pregnancy and exposure to certain bacteria or medications. The most common drugs thought to be involved are bactrim, minocycline, granulocyte colony-stimulating factor (G-CSF), and certain blood pressure medications (called angiotensin II receptor antagonists). There are also case reports of reactions after lithium, lasix, hydralazine, tegretol and certain birth control pills.

Diagnosis

The diagnosis is usually made after a small piece of skin is removed (called a biopsy) and examined under the microscope. The dermatopathologist (skin sample expert) looks for increased white blood cells in certain layers of the skin to make the diagnosis. There is a set of criteria that are sometimes used when making a diagnosis. The criteria include: (1) the sudden onset of painful red plaques or nodules, sometimes with clear fluid, and (2) a skin biopsy consistent with the findings listed above. The minor criteria include: (1) periods of general tiredness and fever higher than 101.4 degrees, (2) signs of inflammation on blood tests, (3) prompt and excellent response to treatment with systemic corticosteroids or potassium iodide, (4) illness proceeded by a nonspecific respiratory or gastrointestinal tract infection OR association with inflammatory disease, pregnancy, solid tumors or blood disorders. Because Sweet syndrome can be associated with other diseases, it is important to evaluate for the associated diseases, though these are extremely rare in children.

Treatment

In the majority of cases, steroids are rapidly effective. High doses are usually continued for four weeks and then slowly decreased over time. Relapses may occur at this point, especially when the steroids are decreased too quickly. If this occurs, the steroids are increased to their original dose and their dosage is reduced over a longer time period. Patients sometimes require maintenance medication to prevent relapses. These long-term medications include indomethacin, colchicine, and potassium iodide, all of which have been reported to be helpful in some patients. Other medications used with varying effects are cyclosporine, dapsone, etretinate, pentoxifylline and clofazimine. If Sweet syndrome is secondary to an underlying disorder, treatment of the disorder in addition to the above medications will result in the most improvement. Given the rarity of the disorder, treatment is very individualized, and requires discussion with a physician who is confident in its treatment.

Prognosis

Prognosis usually depends on the underlying cause. Cases without a known cause seem to have a better outcome than other cases. Disease that responds to medication in a few days will usually completely resolve, however recurrences can occur from 30 to 50% of the time. Relapses are mainly associated with blood disorders or malignancies. Most skin marking resolve without permanent scarring, but the pigment changes could take several months or longer to fade completely.

Connect with other parents

In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.

Weblinks

Sweet Syndrome
Website featuring a discussion forum, new treatments and links to a support group.

Yahoo Support Group
Online link to yahoo support group for individuals with Sweet Syndrome.

http://emedicine.com/derm/topic11.htm
A somewhat technical synopsis of Sweet Syndrome along with a medication summary.

Google Search for Sweet Syndrome

References and Sources

http://emedicine.com/derm/topic11.htm Miller M (2000): Chapter 169: Sweet syndrome in Nelson Textbook of Pediatrics, Behrman RE, Kliegman RM, Jenson HB (eds) 16th Ed, WB Saunders, p 734.