Acute Febrile Neutrophilic Dermatosis

Definition

Definition of Acute Febrile Neutrophilic Dermatosis
Acute febrile neutrophilic dermatosis, also termed Sweet syndrome, is a reactive process characterized by the abrupt onset of tender, red-to-purple papules, and nodules that coalesce to form plaques. The plaques usually occur on the upper extremities, face, or neck and are typically accompanied by fever and peripheral neutrophilia.

Symptoms

Symptoms of Acute Febrile Neutrophilic Dermatosis
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).

Etiology

Etiology of Acute Febrile Neutrophilic Dermatosis
Acute febrile neutrophilic dermatosis may occur with various disorders, including

1. Acute respiratory illness
2. GI infection
3. Cancer
4. Drug exposure
5. Inflammatory or autoimmune disorders
6. Pregnancy

Diagnosis

Diagnosis of Acute Febrile Neutrophilic Dermatosis
The diagnosis of Sweet syndrome is based on both clinical and histopathologic findings. Characteristics that distinguish the lesions of Sweet syndrome from other neutrophilic dermatosis are healing of the lesions without scarring and an absence of vasculitis. However, recent reports suggest that vasculitis should not exclude the diagnosis since it has been shown to occur in many patients with Sweet’s syndrome, which may represent an epiphenomenon instead of a primary immune-mediated process.

Treatment

Treatment of Acute Febrile Neutrophilic Dermatosis
In general, Sweet syndrome responds dramatically to oral corticosteroids and may improve or resolve with treatment of the underlying condition. Without treatment, the syndrome may persist for weeks or months and then improves without leaving scars. Recurrences are common. In rare cases, crops of lesions reappear and the condition persists indefinitely. Cases associated with malignancy can be bullous or ulcerative and resemble atypical pyoderma gangrenosum. These lesions are often recalcitrant to treatment.

Prevention

Prevention of Acute Febrile Neutrophilic Dermatosis
Consult with your doctor.