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Diseases and Conditions
Sweet's syndrome
From MayoClinic.com
Special to CNN.com

Introduction

Sweet's syndrome is a skin disorder characterized by fever and painful skin lesions.

The condition starts suddenly with the appearance of red, slightly raised and tender bumps (plaques), usually on your back, arms, face or neck. These painful bumps rapidly increase in size, and they may progress to blisters.

In most cases, the cause of Sweet's syndrome is undetermined. However, Sweet's syndrome may follow an upper respiratory infection in young adults. Rarely, Sweet's syndrome may occur as a reaction to medication. In older adults, Sweet's syndrome can be associated with certain types of cancer, such as leukemia. Sweet's syndrome occurs most often in women 30 to 50 years of age.

Treatment of Sweet's syndrome, also known as acute febrile neutrophilic dermatosis, is with oral or topical medications, or both.

Signs and symptoms

The distinctive skin lesions are the most obvious sign of Sweet's syndrome.

The lesions seem to appear suddenly as a series of small red bumps that quickly increase in size. The bumps, also called plaques, may grow to be 1 centimeter in diameter or larger. Plaques usually appear on your back, neck, arms and face. These tender and painful eruptions may develop blisters, pustules or ulcers, causing your skin to burn or itch.

The skin lesions may persist for weeks to months and then disappear on their own, even without medication. With medical treatment, you're likely to be free of skin lesions within just a few days.

Sweet's syndrome may be your body's reaction to an internal problem, so it's possible for you to develop the skin lesions at the same time you're experiencing problems with your bones, nervous system, kidneys, intestines, liver, heart, lungs, muscles or spleen. Some of the other specific signs and symptoms of Sweet's syndrome may include:

  • Moderate to high fever
  • Pink eye (conjunctivitis) or sore eyes
  • Tiredness
  • Aching joints and headache
  • Mouth ulcers

Causes

The cause of Sweet's syndrome is usually impossible to determine (idiopathic). But in some cases, it may be your body's immune system reacting to one of the following conditions or circumstances:

  • An upper respiratory tract infection, such as a chest infection or strep throat
  • Blood disorders, including leukemia in older adults (especially acute myelogenous leukemia, a cancer of the blood and bone marrow)
  • Inflammatory bowel disease, such as ulcerative colitis or Crohn's disease
  • Bowel or breast cancer
  • Pregnancy
  • Rheumatoid arthritis
  • An injury at the site where the rash appears, such as from an insect bite or needle prick
  • Certain medications, such as nonsteroidal anti-inflammatory drugs (NSAIDs)

Risk factors

Women are most at risk of Sweet's syndrome, predominately women between the ages of 30 and 50 who have recently had an upper respiratory tract infection. The condition has also been reported in children as young as 3 months and in adults as old as 85.

Pregnant women may get Sweet's syndrome during their first or second trimester. In these cases, the condition usually goes away without medication.

Sweet's syndrome tends to recur. About one-third of people who have had Sweet's syndrome get it again.

When to seek medical advice

Sweet's syndrome is rare. When it occurs, it likely develops after an upper respiratory tract infection. If you develop a red rash that quickly grows in size soon after a bout with strep throat or another upper respiratory infection, see your doctor for appropriate treatment.

Although the rash may eventually disappear without treatment, the right medication can make the rash go away in just a few days. Sweet's syndrome may also be a reaction to a more serious condition, such as an infection or leukemia.

Screening and diagnosis

Your doctor can make a diagnosis of Sweet's syndrome by:

  • The distinctive rash. These red, tender and painful bumps rapidly increase in size and may progress to blisters.
  • Blood tests. Your doctor may take a small sample of blood and have it analyzed to see if you have an unusually high number of white blood cells, which is characteristic of Sweet's syndrome. Your body produces additional white blood cells to defend against inflammation or an infection.
  • Tissue sample. Your doctor may remove a small piece of affected tissue (biopsy) for examination under a microscope. The tissue is analyzed to determine whether it has the characteristic abnormalities of Sweet's syndrome.

Complications

Sweet's syndrome may be a warning sign that your body has a more serious internal problem. For this reason seeking medical care is critical.

For example, Sweet's syndrome sometimes develops as your body's reaction very early in the course of cancer, possibly at a curable stage. Sweet's syndrome may also develop at the same time as other internal problems in your bones, nervous system, kidneys, intestines, liver, heart, lungs, muscles or spleen. Some specific examples of these conditions may include:

  • Inflammatory bowel disease
  • Infections of the upper respiratory tract, including strep throat, pneumonia and tonsillitis
  • Urinary tract infections
  • Infections of the liver and gastrointestinal tract
  • Certain systemic infections

Treatment

Left untreated, Sweet's syndrome not associated with a malignancy may disappear on its own within one to three months. In contrast, the right treatment may improve the skin lesions and associated symptoms dramatically in just two or three days, with the worst of the lesions disappearing within one to four weeks.

With or without treatment, the plaques and lesions rarely leave a mark or scar when they eventually disappear. Your doctor may advise continuing treatment because recurrence of the condition is common.

Medications
Doctors typically prescribe systemic corticosteroids (prednisone or prednisolone) to treat Sweet's syndrome. These oral anti-inflammatory medications lessen redness, itching, swelling and allergic reactions. But there are potential side effects. Corticosteroids may lower your resistance to infections. Also, any infection you get while taking corticosteroids may be harder to treat. Corticosteroids may also cause blurred vision, frequent urination and increased thirst.

Your doctor may also recommend topical corticosteroid treatments to provide direct relief of redness, swelling, itching and discomfort of skin lesions.

In addition, your doctor may suggest that you take nonsteroidal anti-inflammatory drugs (NSAIDs) such as aspirin, ibuprofen or naproxen sodium to reduce inflammation and relieve other signs and symptoms, such as fever and headache.

June 19, 2006

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