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Acute febrile neutrophilic dermatosis - Cellulitis DDx
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Acute febrile neutrophilic dermatosis - 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 may be seen in patients of all ages, but it is most common in healthy women aged 20-60 and individuals with inflammatory bowel disease (IBD) or hematologic malignancies (especially myeloid leukemias and myelodysplastic syndrome). Other common associations include pregnancy, streptococcal pneumonia, autoimmune disorders, radiation therapy, lymphedema, and medications (sulfamethoxazole-trimethoprim, minocycline, granulocyte colony-stimulating factor). Most cases are idiopathic or associated with benign conditions; about 15%-20% are associated with malignancy. 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. In children, Sweet syndrome has also been reported in chronic granulomatous disease and periodic fever syndromes.

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.

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

Neutrophilic dermatosis of the dorsal hands is considered by many to represent a localized form of Sweet syndrome. Hematologic malignancies and IBD are the most common associations with this subtype.

Necrotizing neutrophilic dermatosis describes a subset of patients with severe Sweet syndrome or pyoderma gangrenosum who develop, in addition to their cutaneous disease, fever, leukocytosis (or a leukemoid reaction), and features of shock. Skin pain is a prominent symptom. Additionally, soft tissues underlying areas of skin involvement may be affected with neutrophilic infiltrates and necrosis. This has only been described in adults thus far.

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

Sweet syndrome can be differentiated from cellulitis based on associated symptoms and the presence of numerous scattered lesions.

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

Additional diagnoses to consider in pediatric patients:

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 - Cellulitis DDx
See also in: Overview
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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.
Copyright © 2019 VisualDx®. All rights reserved.