van der Hulst R W, Tieben L M, Gerritsen J J, Bruijn J A, Kluin-Nelemans J C
Afd. Algemene Interne Geneeskunde, Academisch Ziekenhuis, Leiden.
Ned Tijdschr Geneeskd. 1993 Dec 25;137(52):2701-5.
The clinical presentation and the relation with malignancy of acute febrile neutrophilic dermatosis in 7 patients are described. The syndrome is characterised by fever, neutrophilic granulocytosis, painful erythematous plaques and typical histology consisting of a dense dermal infiltrate of mature neutrophils. The idiopathic form (80% of reported patients) usually follows a viral infection. The secondary form (20% of reported patients) is associated with a malignancy, which in the large majority (85%) is a hematologic disorder (this was the case in 3 of the 7 patients); the course of the syndrome may then be serious. The secondary form of the disease may be associated with an exacerbation of a preexistent malignancy, as was seen in 2 of the 3 patients. If the syndrome runs a serious course the treatment consists of corticosteroid administration.
本文描述了7例急性发热性嗜中性皮病患者的临床表现及其与恶性肿瘤的关系。该综合征的特征为发热、嗜中性粒细胞增多、疼痛性红斑斑块以及典型的组织学表现,即真皮层有密集的成熟嗜中性粒细胞浸润。特发性形式(占报告病例的80%)通常在病毒感染后出现。继发性形式(占报告病例的20%)与恶性肿瘤相关,其中绝大多数(85%)为血液系统疾病(本研究的7例患者中有3例为此情况);此时综合征的病程可能较为严重。疾病的继发性形式可能与既往存在的恶性肿瘤病情加重有关,本研究的3例患者中有2例出现这种情况。如果综合征病程严重,治疗方法为给予皮质类固醇。