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Palisaded neutrophilic and granulomatous dermatitis

Author
Adela Rambi G Cardones, MD
Section Editor
Jeffrey Callen, MD, FACP, FAAD
Deputy Editor
Abena O Ofori, MD

INTRODUCTION

Palisaded neutrophilic and granulomatous dermatitis (PNGD) is an inflammatory cutaneous disorder of unknown etiology that usually manifests as skin-colored to erythematous papules or plaques on the extremities (picture 1). PNGD typically occurs in association with systemic disease. Rheumatoid arthritis and systemic lupus erythematosus are the most common associated diseases.

The clinical features, diagnosis, and management of PNGD will be reviewed here. Other neutrophilic dermatoses are reviewed separately. (See "Neutrophilic dermatoses".)

HISTORY

The term PNGD was first proposed in 1994 in a report describing a papular eruption on the extremities in several patients with systemic lupus erythematosus, rheumatoid arthritis, or uncharacterized collagen vascular disease [1]. Biopsies of involved skin revealed histopathologic findings consistent with an evolving immune complex disorder (see 'Histopathology' below). Prior cases of PNGD were classified under other names, including Churg-Strauss granuloma [2], linear subcutaneous bands [3], rheumatoid papules [4], superficial ulcerating rheumatoid necrobiosis [5], and cutaneous extravascular necrotizing granuloma of Winkelmann or Wilkelmann granuloma [6].

EPIDEMIOLOGY

PNGD is rare. A 2008 literature review identified only 97 reported cases [7]. PNGD usually develops in adults; rarely, children are affected [7,8]. Women are affected more frequently than men, an observation that may be related to the association of PNGD with autoimmune diseases [7]. (See 'Associated disorders' below.)

PATHOGENESIS

The pathogenesis of PNGD is unknown. Proposed theories for etiopathogenesis include abnormal neutrophil activation, circulating immune complex deposition, a delayed-hypersensitivity-type reaction, and a low-grade, small-vessel vasculitis [8]. Some authors have proposed that the clinical and histologic features of PNGD are manifestations of an evolving immune complex-mediated process [1]. In accordance with this theory, PNGD may represent an underlying leukocytoclastic vasculitis that acquires granulomatous features as the condition progresses [1,7].

        

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Literature review current through: Nov 2016. | This topic last updated: Sun Dec 13 00:00:00 GMT+00:00 2015.
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References
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