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Sweet syndrome in Child
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Sweet syndrome in Child

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Contributors: Andrew Walls MD, Susan Burgin MD
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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 but is seen more frequently as patients approach adulthood.

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 G-CSF), 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.

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 (GI) 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, Sweet syndrome has been considered a dysregulated cytokine response associated with inflammatory, infectious, and malignant disease. It demonstrates pathergy and will arise in sites of cutaneous injury, such as needle sticks.

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

Codes

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

SNOMEDCT:
84625002 – Sweet's syndrome

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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: 11/18/2016
Last Updated: 01/16/2017
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Sweet syndrome in Child
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Sweet syndrome : Fever, Painful skin lesions, CRP elevated, ESR elevated, WBC elevated, Smooth nodules, Smooth plaques
Clinical image of Sweet syndrome
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