The Neutrophilic Dermatoses

David Farhi, MD; Daniel Wallach, MD


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

In This Article

Abstract and Introduction

The neutrophilic dermatoses (ND) may present with pustules, plaques, ulcerations, and general malaise. They are secondary to the invasion of the skin by normal polymorphonuclear leukocytes in the absence of infection. ND are often associated with particular systemic diseases. The management of affected patients is reviewed, with a focus on nursing care.

Neutrophilic dermatoses (ND) are a group of diseases characterized by the presence of a non-infectious infiltrate of mature neutrophilic leukocytes in the skin (Wallach & Vignon-Penna men, 2006). The three main other features of ND are (a) the potential occurrence of extracutaneous neutrophilic infiltrates; (b) the frequent association with some peculiar systemic diseases; and (c) the possibility of an overlap between several ND.

The group of ND represents a modern conception encompassing several different dermatoses. The first ND identified was erythema elevatum diutinum in 1894 (Crocker & Williams, 1894). Since then, several other ND have been described, in cluding Sweet's syndrome (or "acute febrile neutrophilic dermatosis"), the prototypic ND. One can expect that others will be individualized in the future. Thus, ND is an evolving concept. The ND are classified according to their clinical and histological characteristics (see Table 1 ).

It should be emphasized that even though elevated neutrophils (either in tissues or in the blood) are frequently associated with infectious — mostly bacterial — processes, the concept of ND refers to diseases where infections have no recognized direct role. This fact is reflected by three common features of ND: (a) all skin and blood cultures are negative for infectious agents; (b) ND are not contagious diseases; and (c) antibiotics have no effects on the evolution of ND. Nevertheless, it is a repeatedly seen situation that fever, pus (or pustules), and elevated blood neutrophils, which may be variably associated in genuine ND, lead patients to be inadequately treated with antibiotics. Indeed, the first differential diagnoses of ND are bacterial infections.

The mechanism of neutrophilic invasion of skin and other tissues in the absence of infection remains unclear. The infiltrating neutrophils are normal. It is postulated that they are attracted in tissues by inflammatory cytokines and growth factors. As a consequence of the absence of identified pathophysiology, all current treatments are symptomatic and flares may occur upon treatment withdrawal.

In this article the diagnostic aspects of the main neutrophilic dermatoses are reviewed, and the management of affected patients outlined, with a particular focus on the nursing care aspects.


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