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早期皮肤活检有助于新生儿和婴儿红皮病的诊断和治疗。

Early skin biopsy is helpful for the diagnosis and management of neonatal and infantile erythrodermas.

作者信息

Leclerc-Mercier S, Bodemer C, Bourdon-Lanoy E, Larousserie F, Hovnanian A, Brousse N, Fraitag S

机构信息

Department of Pathology, Necker-Enfants Malades Hospital, APHP, Université René-Descartes, Paris V, France.

出版信息

J Cutan Pathol. 2010 Feb;37(2):249-55. doi: 10.1111/j.1600-0560.2009.01344.x. Epub 2009 Jul 13.

Abstract

BACKGROUND

Erythrodermas are often life-threatening conditions in infants. Determination of the underlying cause is crucial. Microscopic changes in adult erythroderma lack specificity.

OBJECTIVE

To determine if an early skin biopsy is helpful for the diagnosis of neonatal and infantile erythroderma.

METHODS

Seventy-two patients admitted for erythroderma in the first year of life were retrospectively included. One hundred and eleven skin biopsies (12-year period) were examined by 3 pathologists blinded to the clinical diagnosis, and classified into atopic dermatitis, immunodeficiency (ID), psoriasis, Netherton syndrome (NS), ichthyosis, other. From year 2000, LEKTI antibody was performed when NS was suspected. Pathological diagnosis was then compared with clinical diagnosis.

RESULTS

The final diagnosis was made in 69.3% of the cases. In 57.6%, pathological diagnosis was in accordance, and in 11.7%, it was in accordance, but other diagnosis had also been proposed. For ID, sensitivity and specificity were 58.5 and 98.5%, respectively. Before year 2000, NS was frequently misdiagnosed with psoriasis, but with the use of LEKTI antibody, sensitivity and specificity were 100%.

CONCLUSION

Skin biopsy is helpful for etiologic diagnosis of early erythroderma of infancy, particularly in ID and NS, the most severe diseases. Consequently, these results justify an early systematic skin biopsy for a better and earlier management.

摘要

背景

红皮病在婴儿中通常是危及生命的疾病。确定潜在病因至关重要。成人红皮病的微观变化缺乏特异性。

目的

确定早期皮肤活检是否有助于新生儿和婴儿红皮病的诊断。

方法

回顾性纳入72例出生后第一年因红皮病入院的患者。3名对临床诊断不知情的病理学家检查了111份皮肤活检标本(12年期间),并将其分为特应性皮炎、免疫缺陷(ID)、银屑病、Netherton综合征(NS)、鱼鳞病、其他。从2000年起,怀疑为NS时进行LEKTI抗体检测。然后将病理诊断与临床诊断进行比较。

结果

69.3%的病例做出了最终诊断。57.6%的病例病理诊断一致,11.7%的病例病理诊断一致,但也提出了其他诊断。对于ID,敏感性和特异性分别为58.5%和98.5%。2000年前,NS常被误诊为银屑病,但使用LEKTI抗体后,敏感性和特异性均为100%。

结论

皮肤活检有助于婴儿早期红皮病的病因诊断,特别是在ID和NS这两种最严重的疾病中。因此,这些结果证明早期系统性皮肤活检有助于更好、更早地进行治疗。

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