Acute Febrile Neutrophilic Dermatosis

Sweet Syndrome (Acute Febrile Neutrophilic Dermatosis): an inflammatory skin condition that manifests with sudden-onset of fever & skin lesions (papules, plaques & nodules). Associated with infections (GI, respiratory), malignancy (AML), pregnancy, and certain medications.

A biopsy sample of the ulcer showed a dense neutrophilic infiltrate extending to the base of the lesion, a finding consistent with a neutrophilic dermatosis.

The lesions abated with methylprednisolone and oral cyclosporine therapy, and wound care. At follow-up 12 months later, most of the lesions had almost completely resolved, with no evidence of ongoing inflammation.

Treatment of acute febrile neutrophilic dermatosis usually results in rapid improvement in symptoms. Usually, systemic steroids, such as prednisolone, are prescribed in a dose of 30–60 mg daily. Within a few days the fever, skin lesions and other symptoms clear up. However, lower doses of corticosteroids are often required for several weeks to months to prevent relapse.

Several other medications may be tried when systemic corticosteroids are ineffective or contraindicated. Those reported to be useful include:

    Topical corticosteroids

    Intralesional corticosteroids

    Colchicine

    Potassium iodide solution

    Dapsone

    Non-steroidal anti-inflammatory drugs (indomethacin)

    Ciclosporin

    Anakinra

    Other biologic agents such as infliximab, adalimumab or etancercept

    Minocycline

    Mycophenolate

    Clofazimine

    Thalidomide

In some cases, acute febrile neutrophilic dermatosis is very resistant to treatment.