Acute febrile neutrophilic dermatosis in Infant/Neonate
See also in: Cellulitis DDxAlerts and Notices
Synopsis
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
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Bacterial infections (furunculosis, cellulitis)
- Sporotrichosis
- Mycobacteria sp. infections (typical and atypical) including Mycobacterium marinum
- Majocchi granuloma
- Erythema nodosum – Primarily over the shins.
- Acute hemorrhagic edema of infancy
- Pyoderma gangrenosum – Begins as a pustule and evolves into a purulent ulcer with rolled borders. Has similar associations with IBD and malignancy.
- Neutrophilic eccrine hidradenitis – Drug-induced toxicity of the eccrine coils 1-2 weeks following chemotherapy. Palmar involvement is more suggestive of this diagnosis. Patients typically lack fevers but pathology may be required to differentiate these entities.
- CANDLE syndrome
- Erythema multiforme – Should display classic target lesions with 3 zones of color, favors acral surfaces. Oral involvement common.
- Bowel-associated dermatosis-arthritis syndrome – Pustular skin lesions and aseptic arthritis in bowel-bypass patients.
- Majeed syndrome – Multifocal osteomyelitis, congenital anemia, and Sweet syndrome-like skin dermatosis.
- Chronic granulomatous disease
- Wells syndrome – Involved skin is typically neither tender nor warm. Eosinophilia present in 50%, neutrophilia would be unusual. Eosinophils and flame figures seen on pathology.
- Drug eruption
- Azathioprine hypersensitivity syndrome – Acute neutrophilic and systemic reaction after initiating azathioprine.
- Urticarial vasculitis
- Erythema elevatum diutinum – Primarily over extensor surfaces.
- Cutaneous small vessel vasculitis – Presents symmetrically on lower extremities, typically smaller than the plaques of Sweet syndrome.
- Behçet disease – Rare, recurrent, and associated with oral or genital ulcers.
- Bromoderma or iododerma
- Leishmaniasis (New World and Old World) – Recent travel to endemic areas.
- Lymphoma / leukemia cutis
- Metastatic carcinoma
- Sarcoidosis
- Orf
- Cat-scratch disease
- Cutaneous anthrax
- Coccidioidomycosis
- VEXAS syndrome
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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.Subscription Required
References
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Last Reviewed:02/05/2019
Last Updated:07/26/2021
Last Updated:07/26/2021
Acute febrile neutrophilic dermatosis in Infant/Neonate
See also in: Cellulitis DDx