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[表现为水痘感染的先天性朗格汉斯细胞组织细胞增多症]

[Congenital Langerhans-cell histiocytosis presenting as a varicella infection].

作者信息

Girisch M, Fartasch M, Schroth M, Rauch R, Leipold G, Franz K, Heininger U

机构信息

Klinik mit Poliklinik für Kinder and Jugendliche, Universität Erlangen-Nürnberg.

出版信息

Klin Padiatr. 1999 Sep-Oct;211(5):403-5. doi: 10.1055/s-2008-1043820.

Abstract

UNLABELLED

We report on a preterm infant (33rd gestational week) with a varicella-like congenital rash, which initially appeared to respond to therapy with acyclovir. At the age of 3 weeks, lesions were in different stages of evolution and still resembled a varicella zoster virus (VZV) infection. However, since proof of VZV infection was lacking and new lesions erupted at the age of 4 weeks, a skin biopsy was performed which revealed a diagnosis of Langerhans cells histiocytosis. Therapy with prednisone resulted in prompt healing of the lesions.

DISCUSSION

Congenital Langerhans cell histiocytosis is rare and symptoms may vary substantially from case to case. Like in our observation it may be confused with congenital varicella. In case of congenital skin lesions of uncertain etiology a skin biopsy should be performed.

摘要

未标注

我们报告一例早产婴儿(孕33周),患有类似水痘的先天性皮疹,最初似乎对阿昔洛韦治疗有反应。3周龄时,皮损处于不同的演变阶段,仍类似水痘带状疱疹病毒(VZV)感染。然而,由于缺乏VZV感染的证据,且在4周龄时出现新的皮损,遂进行皮肤活检,结果显示诊断为朗格汉斯细胞组织细胞增生症。泼尼松治疗使皮损迅速愈合。

讨论

先天性朗格汉斯细胞组织细胞增生症罕见,症状在不同病例中可能有很大差异。如我们的观察所示,它可能与先天性水痘相混淆。对于病因不明的先天性皮肤损害,应进行皮肤活检。

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