Acute febrile neutrophilic dermatosis (Sweet Syndrome)

Proper Name: Acute febrile neutrophilic dermatosis

Common Name: Sweet Syndrome, Sweet disease, Sweet's syndrome, Sweet's disease, neutrophilic dermatitis, granulocyte colony-stimulating factor, G-CSF

Age Onset: Although infants and older-aged adults can develop Sweet Syndrome, it most commonly affects women between the ages of 30 and 50.

Duration: Lesions from Sweet Syndrome may last anywhere from a few days to over four years, and reoccurance is common.

Males/Females/Equal: Females are more prone to have Sweet Syndrome than males.

Particular Ethnic Group: Sweet Syndrome is very rare and has no known ethnic inclinations.

Cause: Exact cause is not known, but it can also develop early in the onset of cancer. Since Sweet Syndrome cannot be targeted for a specific cause, the syndrome can sometimes be an immune response:

• to an upper respiratory tract infection (such as a chest infection or strep throat),
• to blood disorders (esp. acute myelogenous leukemia),
• to inflammatory bowel disease (such as ulcerative colitis or Crohn's disease),
• to bowel or breast cancer,
• to pregnancy,
• to Rheumatoid arthritis,
• to vaccination, and
• to Certain medications (such as nonsteroidal anti-inflammatory drugs (NSAIDs)).

Symptoms:

• distinctive skin lesions, that develop typically to this pattern: 1st.) a series of small red bumps appear suddenly on your back, neck, arms and face, often after a fever or upper respiratory infection, 2nd.) the bumps grow quickly in size, spreading into clusters called plaques that may be a centimeter in diameter or larger, 3rd.) the eruptions that are tender or painful, may develop blisters, pustules or even ulcers, and 4th.) lesions may persist for weeks to months and disappear on their own, without medication; with medical treatment the lesions coud be gone in a few days.
• Moderate to high fever
• Pink eye (conjunctivitis) or sore eyes
• Tiredness
• Aching joints and headache
• Mouth ulcers

Prognosis (describe the progression of the disease): Sweet Syndrome begins as a series of small red bumps appearing suddenly on your back, neck, arms and face, often after a fever or upper respiratory infection. These bumps grow rapidly in size, spreading into clusters called plaques that may be a centimeter in diameter or larger. The eruptions are tender or painful and may develop blisters, pustules or even ulcers. Since Sweet Syndrome is commonly but not always accompanied by a more serious disease, the spread of the serious disease is also a progession of Sweet Syndrome.

Treatment Options (Rx):

1. If the lesions are left untreated and Sweet syndrome is not associated with another condition, the lesions may disappear within 1 to 3 months.
2. Medications can improve the skin lesions in a couple days to the worst cases within 1 to 4 weeks. Doctors usually prescribe systemic corticosteroids (prednisone or prednisolone) to treat Sweet syndrome because these oral anti-inflammatory medications reduce redness, itching, swelling and allergic reactions. But long-term use of corticosteroids can cause serious side effects, including an increased risk of hard-to-treat infections, osteoporosis, eye problems and diabetes. Topical corticosteroids may be used to provide immediate relief of swelling. 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. It is not unheard of for doctors to continue the treatment for Sweet Syndrome because it can reoccur.
3. Avoid injury to the skin and wear protective clothing.
4. Apply sunscreen with a sun protection factor (SPF) of 15 or greater before heading outdoors.
   

Links:

<http://www.mayoclinic.com/print/sweets-syndrome/DS00752/DSECTION=all&METHOD=print>

<http://dermatology.cdlib.org/DOJvol5num1/therapy/sweets.html>

<http://dermnetnz.org/reactions/sweets.html>

<http://www.emedicine.com/derm/TOPIC11.HTM>