Sweet's Syndrome - Acute Febrile Neutrophilic Dermatosis

DEFINITION

Sweet's syndrome — also known as acute febrile neutrophilic dermatosis — is a rare skin condition marked by fever and painful skin lesions that appear mainly on your arms, neck, face and back.

The exact cause of Sweet's syndrome isn't always known. In some people, it's triggered by an infection, illness or certain medications. Sweet's syndrome can also occur with some types of cancer.

The most common treatment for Sweet's syndrome is corticosteroid pills, such as prednisone. Signs and symptoms often disappear just a few days after treatment begins, but recurrence is common.

Sweet's Syndrome

SYMPTOMS

Sweet's syndrome is marked by an abrupt eruption of small red bumps on your arms, neck, face or back — often after a fever or upper respiratory infection. The bumps grow quickly in size, spreading into painful clusters up to an inch or so in diameter.

When to see a doctor

If you develop a painful, red rash that quickly grows in size, see your doctor for appropriate treatment.

CAUSES

In most cases, the cause of Sweet's syndrome isn't known. Sweet's syndrome is sometimes associated with cancer, most often leukemia. A few cases may be associated with a solid tumor, such as breast or colon cancer. Sweet's syndrome may also occur as a reaction to a medication — most commonly a type of drug that boosts production of white blood cells.

RISK FACTORS

Sweet's syndrome is uncommon, but certain factors increase your risk, including:

  • Your sex. Women are more likely to have Sweet's syndrome than men.
  • Your age. Though older adults and even infants can develop Sweet's syndrome, the condition mainly affects women between the ages of 30 and 50.
  • Cancer. Sweet's syndrome is sometimes associated with cancer, most often leukemia. A few cases may be associated with a solid tumor, such as breast or colon cancer.
  • Other health problems. Sweet's syndrome often follows an upper respiratory infection, and many people report having flu-like symptoms before the rash appears. Sweet's syndrome can also be associated with inflammatory bowel disease.
  • Pregnancy. Some women develop Sweet's syndrome during pregnancy. In these cases, the condition usually clears without treatment.

COMPLICATIONS

There is a risk of the skin lesions becoming infected. Follow your doctor's recommendations for caring for the affected skin.

In cases where Sweet's syndrome is associated with cancer, the eruptions of the lesions may be the first sign of cancer either appearing or recurring.

TESTS AND DIAGNOSIS

Your dermatologist can usually diagnose Sweet's syndrome simply by looking at the lesions. But you're likely to have certain tests to rule out conditions that have similar symptoms and to search for the underlying cause. These tests include:

  • Blood tests. A sample of your blood may be sent to a laboratory where it's checked for an unusually large number of white blood cells and certain blood disorders.
  • Skin biopsy. Your doctor may remove a small piece of affected tissue for examination under a microscope. The tissue is analyzed to determine whether it has the characteristic abnormalities of Sweet's syndrome.

TREATMENTS AND DRUGS

In some cases, Sweet's syndrome resolves without treatment. But medications can speed the process dramatically. The most common medications used for Sweet's syndrome are corticosteroids, which come in a variety of forms, including:

  • Pills. Oral corticosteroids, such as prednisone, work very well but will affect your entire body. Long-term use can cause weight gain and weakened bones.
  • Creams or ointments. These preparations usually affect just the portion of skin where they're applied, but can cause thinning skin.
  • Injections. Another option is to inject a small amount of corticosteroid right into each lesion. This may be less feasible for people who have a great number of lesions.

To avoid the side effects associated with long-term corticosteroid use, your doctor may suggest other types of oral medications, including:

  • Dapsone
  • Potassium iodide
  • Colchicine
  • Indomethacin
  • Clofazimine
  • Cyclosporine

LIFESTYLE AND HOME REMEDIES

If you have Sweet's syndrome, it's important to treat your skin gently. These steps can help reduce additional injury to the skin:

  • Avoid injury to your skin. Wear protective clothing if you think you might injure or damage your skin.
  • Apply sunscreen. Use sunscreen with a sun protection factor (SPF) of 15 or greater before you head outdoors.

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