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Neutrophilic dermatoses

Samuel L Moschella, MD, FAAD, FACP
Section Editor
Jeffrey Callen, MD, FACP, FAAD
Deputy Editor
Abena O Ofori, MD


The neutrophilic dermatoses are a group of disorders characterized by skin lesions for which histologic examination reveals intense epidermal, dermal, or hypodermal infiltrates composed primarily of neutrophils with no evidence of infection or true vasculitis [1]. Classification of the neutrophilic dermatoses is based upon the recognition of clinical and pathologic features, as well as the identification of associated diseases [1,2].

Cutaneous findings in neutrophilic dermatoses are variable, and can include vesiculopustules, plaques, nodules, or ulcerations (table 1) [1,3,4]. Depending on the disorder, lesions may be localized or widespread. Extracutaneous involvement may be present in some cases [5].

The pathogenic mechanisms of the various neutrophilic dermatoses are not well understood. The occurrence of neutrophilic dermatoses in autoinflammatory diseases and observed similarities in the clinical and histologic features, cytokine profiles, and therapeutic approaches between neutrophilic dermatoses and autoinflammatory diseases have led some authors to propose that neutrophilic dermatoses should be considered autoinflammatory diseases [6]. (See "Periodic fever syndromes and other autoinflammatory diseases: An overview".)


The prototype of the neutrophilic dermatoses is Sweet syndrome (acute febrile neutrophilic dermatosis). There are four primary features of Sweet syndrome: a cutaneous eruption consisting of erythematous papules and plaques, a dermal nonvasculitic neutrophilic infiltration on biopsy, fever, and peripheral neutrophilia [7,8]. Rare clinical variants include cellulitis-like, subcutaneous, and necrotizing fasciitis-like presentations [9-11].

The cutaneous eruption consists of erythematous to violaceous tender papules which enlarge to form plaques with irregular pseudovesicular surfaces (picture 1A-D). True pustulation and blistering also can occur. The plaques are usually a few centimeters in diameter, and may have central yellowish discoloration, creating a target-like appearance. The plaques may cause pain and a burning sensation, but are not pruritic.

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Literature review current through: Nov 2017. | This topic last updated: Jul 24, 2017.
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