Rare Pulmonology News

Disease Profile

Acute febrile neutrophilic dermatosis

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

Adult

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ICD-10

L98.2

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Sweet syndrome; SS; Neutrophilic dermatosis, acute febrile;

Categories

Musculoskeletal Diseases; Skin Diseases

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.[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]

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.[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]

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Medical Terms Other Names
Learn More:
HPO ID
1%-4% of people have these symptoms
Acne inversa
0040154
Anemia
Low number of red blood cells or hemoglobin
0001903
Arthralgia
Joint pain
0002829
Cystic acne
0033188
Dilated cardiomyopathy
Stretched and thinned heart muscle
0001644
Elevated C-reactive protein level
0011227
Elevated erythrocyte sedimentation rate
High ESR
0003565
Myalgia
Muscle ache
Muscle pain

[ more ]

0003326
Panniculitis
Inflammation of fat tissue
0012490
Pyoderma gangrenosum
0025452
Recurrent fever
Episodic fever
Increased body temperature, episodic
Intermittent fever

[ more ]

0001954
Percent of people who have these symptoms is not available through HPO
Autosomal dominant inheritance
0000006
Childhood onset
Symptoms begin in childhood
0011463
Erythema
0010783
Increased circulating interleukin 6
0030783
Small vessel vasculitis
0011944

Cause

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)

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.[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]

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

Organizations Supporting this Disease

    Learn more

    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.

        References

        1. Sweet's syndrome. MayoClinic.com. December 10, 2015; https://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; https://www.dermnetnz.org/topics/acute-febrile-neutrophilic-dermatosis/.
        4. Yoon-Soo (Cindy) Bae, Sharon A Salter. Acute Febrile Neutrophilic Dermatosis. Medscape. March 31, 2016; https://emedicine.medscape.com/article/1122152.

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