Summary

Acute febrile neutrophilic dermatosis is a skin condition characterized by fever, inflammation of the joints (arthritis), and painful skin lesions that appear mainly on the face, neck, back and arms. 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. It is suspected that it can be a reaction of the body to certain exposures (sun), infections (Streptococcus bacteria, Campylobacter), or medications (azathioprine, nonsteroidal anti-inflammatory 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, such as corticosteroid use.

Sign of Sweet Syndrome

Bumps on the Skin

People often describe localized swollen areas on, or under, the skin as lumps or bumps. While bumps on, or under, the skin may result from conditions that give rise to a skin rash, many other conditions can result in solitary raised lumps on the skin. Infections, tumors, and the body's response to trauma or injury can all lead to lumps or bumps that appear to be located on or underneath the skin.

Depending upon the cause, skin lumps or bumps may vary in size and be firm or soft to the touch. The overlying skin may be reddened or ulcerated. Skin bumps may or may not be painful or tender, depending upon the cause of the lesions.

Symptoms

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:

Sometimes other areas of the body are affected, such as the bones, nervous system, kidneys, intestines, liver, heart , lungs, muscles, and spleen.

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Treatment

Left untreated, acute febrile neutrophilic dermatosis not associated with a more serious condition may disappear on its own within one to three months. Medications, such as corticosteroids (prednisone or prednisolone), 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 (which are taken by mouth) to treat this condition. These medications reduce redness, itching, swelling and allergic reactions.

If an underlying cause can be identified, it should be treated (i.e. surgical removal of solid tumors, treatment of infections, or discontinuation of medications). Successful therapy of the underlying disorder may promote resolution of acute febrile neutrophilic dermatosis and prevent recurrences.

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.

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Find a Specialist

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can't find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

Healthcare Resources

  • To find a medical professional who specializes in genetics, you can ask your doctor for a referral or you can search for one yourself.

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group's website or contact them to learn about the services they offer.

Organizations Supporting This Disease

Sweet's Syndrome UK
168 Tweedsmuir Road
Tremorfa, Cardiff, CF24 2RG United Kingdom
E-mail: [email protected]
Website: https://helpforsweetssyndromeuk.wordpress.com/

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References
SOURCE:

United States. National Institutes of Health. Genetic and Rare Diseases Information Center. "Acute Febrile Neutrophilic Dermatosis." Nov. 4, 2016. <https://rarediseases.info.nih.gov/diseases/521/acute-febrile-neutrophilic-dermatosis>.
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