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Genetic and Rare Diseases Information Center (GARD)

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Acute febrile neutrophilic dermatosis


Other Names for this Disease

  • Gomm Button disease
  • Neutrophilic dermatosis, acute febrile
  • SS
  • Sweet syndrome
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

I have a young friend who is affected by acute febrile neutrophilic dermatosis (Sweet syndrome). Can you provide me with information about this condition that can be shared with her physicians?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is acute febrile neutrophilic dermatosis?

Acute febrile neutrophilic dermatosis - also known as Sweet syndrome - is a skin condition marked by fever, inflammation of the joints (arthritis), and painful skin lesions that appear mainly on the face, neck, back and arms.[1][2] Although middle-aged women are most likely to develop this condition, it may also affect men, older adults and even infants. The exact cause of acute febrile neutrophilic dermatosis often isn't known. In some people, it's triggered by an infection, illness or certain medications. This condition can also occur with some types of cancer and other serious health problems. Most often, it isn't serious and will clear on its own in a few months. Healing is much more rapid, however, with treatment.[1]
Last updated: 7/23/2009

What signs and symptoms are associated with acute febrile neutrophilic dermatosis?

The most obvious signs of acute febrile neutrophilic dermatosis are distinctive skin lesions that usually develop according to a specific pattern. Typically, a series of small red bumps appear suddenly on the back, neck, arms and face, often after a fever or upper respiratory infection. The bumps grow quickly 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. Lesions may persist for weeks to months and then disappear on their own, without medication. With medical treatment, the skin lesions may resolve in just a few days.[1]

Other signs and symptoms of acute febrile neutrophilic dermatosis may include:[1]

  • Moderate to high fever
  • Pink eye (conjunctivitis) or sore eyes
  • Tiredness
  • Aching joints and headache
  • Mouth ulcers
Last updated: 7/23/2009

What causes acute febrile neutrophilic dermatosis?

In many cases, the cause of acute febrile neutrophilic dermatosis is unknown (idiopathic).[1][2] But sometimes, it can be a sign of an immune system response to one of the following:[1]

  • An upper respiratory tract infection, such as a chest infection or strep throat
  • Blood disorders, 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 an insect bite or needle prick
  • Certain medications, including nonsteroidal anti-inflammatory drugs (NSAIDs)
Last updated: 7/23/2009

How might acute febrile neutrophilic dermatosis be treated? 

Left untreated, acute febrile neutrophilic dermatosis not associated with a more serious condition may disappear on its own within one to three months. Medications can improve skin lesions and associated symptoms in just two or three days, with the worst of the lesions disappearing within one to four weeks. Doctors usually prescribe systemic corticosteroids (prednisone or prednisolone) to treat this condition. These oral anti-inflammatory medications reduce redness, itching, swelling and allergic reactions.[1] 

In the pediatric population, long-term use of corticosteroids can cause problems with linear growth, blood pressure, and blood glucose levels. Children may also have social sequelae associated with their use. Therefore, attempts are usually made to treat children with steroid-sparing drugs. Other treatment options include indomethacin, colchicine, potassium iodidedapsone, cyclosporine, etretinate, pentoxifylline, clofazimine, doxycycline, metronidazole, isotretinoin, methotrexate, cyclophosphamide, chlorambucil, and interferon alpha, all of which have shown some success in the resolution of symtpoms.[3]  

With or without treatment, the lesions rarely leave a mark or scar when they eventually disappear. Even after the lesions have resolved, treatment may continue, as recurrence of the condition is common.[1]

If an underlying cause can be identified, it should be treated (i.e. resection of solid tumors, treatment of infections, and discontinuation of causative medication). Successful therapy of the underlying disorder may promote resolution of acute febrile neutrophilic dermatosis and prevent recurrences.[3]

Last updated: 7/23/2009

What complications may arise in individuals with acute febrile neutrophilic dermatosis?

Although it can be extremely uncomfortable, acute febrile neutrophilic dermatosis often isn't serious. But in some cases, it can be a warning sign of another health problem. For instance, this condition sometimes develops very early in the course of cancer. It may also occur in conjunction with:[1]

  • 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
Last updated: 7/23/2009

References
Other Names for this Disease
  • Gomm Button disease
  • Neutrophilic dermatosis, acute febrile
  • SS
  • Sweet syndrome
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.