National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Acute febrile neutrophilic dermatosis


Other Names:
Sweet syndrome; SS; Neutrophilic dermatosis, acute febrile; Sweet syndrome; SS; Neutrophilic dermatosis, acute febrile; Gomm Button disease See More
Categories:
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.[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. 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.[3][4] 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.[1][4]
Last updated: 11/4/2016
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][3]
  • Moderate to high fever
  • Pink eye (conjunctivitis) or sore eyes
  • Tiredness
  • Aching joints and headache
  • Mouth ulcers
  • Headache
Sometimes other areas of the body are affected, such as the bones, nervous system, kidneys, intestines, liver, heart , lungs, muscles, and spleen.[3]
Last updated: 11/4/2016
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][3][4]
  • 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: 11/4/2016
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.[1][4]

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.[4]

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][4]
Last updated: 11/4/2016

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.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

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. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.
  • The MayoClinic.com provides information about acute febrile neutrophilic dermatosis. Click on the link to access this information.
  • The Merck Manuals Online Medical Library provides information on this condition for patients and caregivers. 
  • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Acute febrile neutrophilic dermatosis. Click on the link to view a sample search on this topic.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new 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? See answer


  1. Sweet's syndrome. MayoClinic.com. December 10, 2015; http://www.mayoclinic.org/diseases-conditions/sweets-syndrome/home/ovc-20165794.
  2. Sweet Syndrome. National Organization for Rare Disorders (NORD). 2015; https://rarediseases.org/rare-diseases/sweet-syndrome/.
  3. Amanda Oakley. Acute febrile neutrophilic dermatosis. DermNet New Zealand. September 2015; http://www.dermnetnz.org/topics/acute-febrile-neutrophilic-dermatosis/.
  4. Yoon-Soo (Cindy) Bae, Sharon A Salter. Acute Febrile Neutrophilic Dermatosis. Medscape. March 31, 2016; http://emedicine.medscape.com/article/1122152.