Acute Febrile Neutrophilic Dermatosis (Sweet Syndrome) Treatment & Management

Updated: Jun 02, 2020
  • Author: Yoon-Soo (Cindy) Bae, MD; Chief Editor: William D James, MD  more...
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Treatment

Medical Care

If identified, successful therapy of the underlying disorder may promote resolution of Sweet syndrome (acute febrile neutrophilic dermatosis) and prevent recurrences. [4, 82] Otherwise, in most cases of Sweet syndrome, prednisone is extremely and rapidly effective, in doses of 0.5-1 mg/kg/day. [2, 6, 28] In more severe presentations, 2 mg/kg/day divided into two doses per day is warranted. [6] Pulmonary infiltrates also tend to respond promptly to prednisone. [90] Despite the excellent response, recurrences of neutrophilic dermatitis are common and generally develop as steroid use is being tapered; therefore, a slow taper is recommended. [6] If the underlying disease flares, it may take longer to effectively taper therapy. [2] High-potency topical steroids (eg, clobetasol propionate 0.05%) or intralesional glucocorticoids (eg, triamcinolone acetonide 3-10 mg/mL) may also be useful in localized lesions. [4]

In the pediatric population, long-term use of corticosteroids can cause problems with linear growth, blood pressure, and blood glucose levels. Children may also have social sequelae associated with their use. Therefore, attempts are usually made to treat children with steroid-sparing drugs. [24] A case of cutis laxa–like lesions in skin previously affected by Sweet syndrome 9 months after presentation and treatment with corticosteroids has been described in an 8-month-old child. [102]

For long-term management, numerous drugs may be helpful. Although the mechanism of many of these medications is inhibition of neutrophil chemotaxis, none has been shown to be more efficacious than corticosteroids. [2, 4]

Indomethacin, colchicine, and potassium iodide were helpful in small series of patients. [2, 112, 113] One retrospective study evaluating 90 patients with acute febrile neutrophilic dermatosis demonstrated the effective use of colchicine as first-line therapy. [114]

Dapsone, cyclosporine, etretinate, pentoxifylline, and clofazimine also have been used, with anecdotal success. [2, 23, 28, 115]

Doxycycline, metronidazole, isotretinoin, methotrexate, cyclophosphamide, chlorambucil, adalimumab, infliximab, intravenous immunoglobulin (IVIG), pulse doses of methylprednisolone, and interferon-alfa are also reportedly successful. [2, 23, 28, 115, 116, 117, 118, 119]

In the treatment of recalcitrant acute febrile neutrophilic dermatosis, thalidomide has been used to clear lesions within 1 month after therapy with corticosteroids, metronidazole, dapsone, and methotrexate failed. [120]

Several reports describe biologic agents successfully treating Sweet syndrome. Etanercept has been reported to control the skin manifestations of acute febrile neutrophilic dermatosis in a small case series of rheumatoid arthritis patients. [121] However, care must be taken when using this treatment in a group with concomitant or a high likelihood of cancer. [122] Anakinra and rituximab have also been reported to be effective in refractory cases. [123, 124] In addition, adalimumab has been reported to successfully treat a recalcitrant case of Sweet syndrome. [125] A more complete list of therapies can be found in a review by Nelson et al. [6]

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Consultations

Consultation with a dermatologist is indicated for the diagnosis and evaluation of underlying causes of Sweet syndrome (acute febrile neutrophilic dermatosis). An internal medicine specialist may be consulted to evaluate any underlying or triggering conditions.

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Prevention

Deterrence is based on avoiding known triggers, such as medications, and treatment of underlying conditions that may be factors.

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Long-Term Monitoring

Outpatient care for the skin lesions of Sweet syndrome (acute febrile neutrophilic dermatosis) is usually coordinated by a dermatologist in conjunction with other physicians who may be involved if other underlying diseases are present.

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