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Neutrophilic dermatoses

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

INTRODUCTION

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 pathogenesis of neutrophilic dermatoses is unknown. It is believed that these disorders represent a state of altered immunologic reactivity. Consistent with this hypothesis is the observation that neutrophilic dermatoses generally respond to systemic glucocorticoids and other immunomodulatory therapies [6].

SWEET SYNDROME

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].

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 2016. | This topic last updated: Tue Apr 07 00:00:00 GMT 2015.
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