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Acute febrile neutrophilic dermatosis in Infant/Neonate
See also in: Cellulitis DDx
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Acute febrile neutrophilic dermatosis in Infant/Neonate

See also in: Cellulitis DDx
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Contributors: Tyler Werbel, Andrew Walls MD, Belinda Tan MD, PhD, Noah Craft MD, PhD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Sweet syndrome, or acute febrile neutrophilic dermatosis, is an inflammatory disorder manifesting as multiple sterile, painful, edematous, erythematous plaques that are usually associated with fever and leukocytosis. The disease is typically skin-limited, although any organ system may also be affected. It is only rarely reported in children and infants. In the pediatric population, the mean age of onset is 5 years, and there is a male predominance, particularly for children under 3 years.

Sweet syndrome is a sterile neutrophilic disorder, and like its counterpart pyoderma gangrenosum, is associated with inflammatory bowel disease (IBD), streptococcal infections, rheumatoid arthritis, medications (typically granulocyte colony-stimulating factor), autoimmune disorders, and hematologic disorders, especially myeloid leukemias and myelodysplastic syndrome. In children, Sweet syndrome has also been reported in chronic granulomatous disease and periodic fever syndromes.

Pathergy is frequently associated, as lesions will arise or worsen in sites of cutaneous injury, such as needle sticks. Fever may accompany or precede cutaneous involvement. The syndrome frequently includes extracutaneous manifestations such as fever, headaches, myalgias, malaise, arthralgias, and ocular inflammation. Other sites that may be affected include the oral mucosa, gastrointestinal tract, lungs, musculoskeletal system, kidneys, heart, and central nervous system (CNS). Hypotension and tachycardia are rare but can occur as a result of systemic inflammation.

Although the exact etiology is still unclear, abnormal cytokine expression and atypical neutrophil function are thought to contribute to the pathogenesis. A genetic predisposition may also be operable.

Sweet syndrome typically responds dramatically to systemic corticosteroids, but recurrences are common.

Codes

ICD10CM:
L98.2 – Febrile neutrophilic dermatosis [Sweet]

SNOMEDCT:
84625002 – Acute febrile neutrophilic dermatosis

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed: 02/06/2019
Last Updated: 03/01/2019
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Acute febrile neutrophilic dermatosis in Infant/Neonate
See also in: Cellulitis DDx
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Acute febrile neutrophilic dermatosis : Fever, Asymmetric trunk or extremity, Painful skin lesions, CRP elevated, ESR elevated, WBC elevated, Smooth nodules, Smooth plaques
Clinical image of Acute febrile neutrophilic dermatosis
A well-demarcated, deep pink, pseudovesicular plaque over the medial clavicle.
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