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Acute febrile neutrophilic dermatosis is characterized by tender, indurated, dark-red papules and plaques with prominent edema in the upper dermis and dense infiltrate of neutrophils. Cause is not known. It frequently occurs with underlying cancer, especially hematologic cancers.
Etiology
Acute febrile neutrophilic dermatosis may occur with various disorders, including
About 25% of patients have an underlying cancer, 75% of which are hematologic cancers, especially myelodysplastic syndromes and acute myeloid leukemia. When not due to cancer, acute febrile neutrophilic dermatosis affects mostly women ages 30 to 50, with a female:male ratio of 3:1. In contrast, men who develop the condition tend to be older (60 to 90).
The cause is unknown; however, type 1 helper T-cell cytokines, including IL-2 and interferon-γ, are predominant and may play a role in lesion formation.
Symptoms and Signs
Patients are febrile, with an elevated neutrophil count, and have tender, dark-red plaques or papules, most often on the face, neck, and upper extremities, especially the dorsum of hands. Oral lesions can also occur. Rarely, bullous and pustular lesions are present. The lesions often develop in crops. Each crop is preceded by fever and persists for days to weeks.
Extracutaneous manifestations can involve the eyes (eg, conjunctivitis, episcleritis, iridocyclitis), joints (eg, arthralgia, myalgia, arthritis), and internal organs (eg, neutrophilic alveolitis; sterile osteomyelitis; psychiatric or neurologic changes; transient kidney, liver, and pancreatic insufficiency).
Diagnosis
Diagnosis is suggested by the appearance of the lesions and is supported by the presence of associated conditions. Differential diagnosis includes erythema multiforme, erythema elevation diutinum, acute cutaneous lupus erythematosus, pyoderma gangrenosum, and erythema nodosum. If diagnosis is unclear, skin biopsy should be done. The histopathologic pattern is that of edema in the upper dermis with a dense infiltrate of neutrophils in the dermis. Vasculitis may be present but is secondary.
Treatment
Treatment involves systemic corticosteroids, chiefly prednisone 0.5 to 1.5 mg/kg po once/day tapered over 3 wk. Antipyretics are also recommended. In difficult cases, dapsone 100 to 200 mg po once/day, indomethacin 150 mg po once/day for 1 wk and 100 mg po once/day for 2 additional wk, or K iodide 900 mg po once/day or 300 mg po tid can be given.
Last full review/revision October 2009 by Wingfield E. Rehmus, MD, MPH
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