The Neutrophilic Dermatoses

David Farhi, MD; Daniel Wallach, MD

Disclosures

Dermatology Nursing. 2008;20(4):274-282. 

In This Article

The Main Neutrophilic Dermatoses

Robert Douglas Sweet described this disease in eight women in 1964, under the explicit term "acute febrile neutrophilic dermatosis" (Sweet, 1964). SS is the most frequent of the ND.

Definition. SS is characterized by a superficial dermal neutrophilic infiltrate, typically with an acute course and high fever.

Clinical signs. Dermatologic signs may be preceded by flu-like symptoms, such as fever (up to 40B0 C), diffuse pain (myalgias, arthralgia), and general malaise. Dermatologic signs typically include elevated papules, nodules, or plaques, sometimes distributed in an annular fashion (see Figure 1). Lesion size varies from a few millimeters to several centimeters. Main locations are the face, neck, and upper limbs, but SS may involve any body site. Atypical forms include bullous, pustular, hemorrhagic, and/or ulcerated lesions. Actually, many of these atypical presentations represent overlap syndromes between SS and other ND.

Figure 1.

Sweet's syndrome

Diagnosis. The diagnosis of SS chiefly relies on the clinical picture and the histopathology of a skin biopsy. Typical SS skin pathology shows an intense infiltration of the superficial dermis by neutrophilic leukocytes, associated with an important edema that may provoke junctional blistering. Dermal vessels are usually normal.

In 20% to 30% of cases, Sweet's syndrome is paraneoplastic, the main associated malignancy being acute leukemia. It is important to rule out such a possibility by blood cell count and appropriate work-up, guided by clinical examination. In rare in stances, SS may be drug induced.

Treatment. Oral steroids, at daily doses of 0.5 to 1 mg/kg are usually rapidly efficient (Cohen & Kurzrock, 2003). Therapeutic successes have been also reported with dapsone and colchicine. For limited forms of SS, topical or intralesional steroids may be used alone or in combination with an oral treatment (Cohen & Kurzrock, 2002).

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