Acute febrile neutrophilic dermatosis
Other Names for this Disease
- Gomm Button disease
- Neutrophilic dermatosis, acute febrile
- Sweet syndrome
Your QuestionI 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?
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Questions on this page
- What is acute febrile neutrophilic dermatosis?
- What signs and symptoms are associated with acute febrile neutrophilic dermatosis?
- What causes acute febrile neutrophilic dermatosis?
- How might acute febrile neutrophilic dermatosis be treated?
- What complications may arise in individuals 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.
Other signs and symptoms of acute febrile neutrophilic dermatosis may include:
- Moderate to high fever
- Pink eye (conjunctivitis) or sore eyes
- Aching joints and headache
- Mouth ulcers
- 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
- 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)
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.
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 iodide, dapsone, 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.
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.
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.
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:
- 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
- Sweet syndrome. MayoClinic.com. 2008; http://www.mayoclinic.com/print/sweets-syndrome/DS00752/DSECTION=all&METHOD=print. Accessed 7/23/2009.
- Sweet Syndrome. National Organization for Rare Disorders (NORD). 2002; http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Sweet%20Syndrome. Accessed 7/23/2009.
- Alavian CN, Salter SA, Boer AF. Acute Febrile Neutrophilic Dermatosis: Treatment & Medication. eMedicine. 2007; http://emedicine.medscape.com/article/1122152-treatment. Accessed 7/23/2009.