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Autumn 2014, Vol. 22 No. 3

Hong Kong J. Dermatol. Venereol. (2014) 22, 115-123


Paediatric Dermatology Column: Review Article

Neutrophilic dermatoses and the concept of autoinflammation from a paediatric perspective

從兒科角度解讀嗜中性白血球增多性皮膚病及其自身炎症的概念

R Toholka and J Su

Abstract

Neutrophilic dermatoses have primarily been classified based on their histological appearance and many have overlapping associations with systemic diseases. This article provides a brief overview of the two most common neutrophilic dermatoses, Sweet's syndrome and pyoderma gangrenosum. The clinical presentation, systemic associations, histology, treatment and the occurrence in the paediatric population of these entities are discussed. A clear understanding of the pathogenesis of these conditions remains elusive. The concept of autoinflammation is introduced, and how further investigation and knowledge of the pathways of the innate immune system may lead to the potential reclassification of neutrophilic dermatoses and more targeted treatment is discussed.

嗜中性白血球增多性皮膚病主要是基於其組織學表現歸類,當中不少與全身性疾病有著重疊的關聯。本文簡要回顧兩種最常見的嗜中性白血球增多性皮膚病:史維特症候群和壞疽性膿皮病,這兩病的臨床表現、全身性關聯、組織學表現、治療方法及在兒童人口中的發病情況將一一探討。現時,嗜中性白血球增多性皮膚病的明確發病機制仍所知甚少。本文將介紹自身炎症這一概念,如何進一步檢查以釐清先天免疫系統的途徑,可能將導致這一類皮膚病的重新分類,並探討更針對性的治療。

Keywords: Inflammasome, paediatric, pyoderma gangrenosum, Sweet's syndrome

關鍵詞: 炎性體、兒科、壞疽性膿皮病、史維特症候群