先天性鱼鳞病样红皮病患儿疾病严重程度评分的建立。

Development of a disease severity score for newborns with collodion membrane.

机构信息

Section of Dermatology, Hospital for Sick Children, and University of Toronto, Toronto, Ontario, Canada.

出版信息

J Am Acad Dermatol. 2014 Mar;70(3):506-11. doi: 10.1016/j.jaad.2013.11.002. Epub 2013 Dec 24.

DOI:10.1016/j.jaad.2013.11.002

PMID:24373778

Abstract

BACKGROUND

Collodion membrane in the neonate may be the initial presentation of a number of different conditions. There is a lack of data correlating the extent of clinical involvement to the underlying disease and prognosis.

OBJECTIVE

We sought to identify features predictive of the final outcome and complications in a cohort of patients with collodion membrane, using a disease severity score.

METHODS

This was a retrospective cohort study of newborns with collodion membrane at a tertiary care institution over a period of 31 years. We designed and applied a 0- to 15-point severity score and correlated the results with the final diagnoses and complications. Data on demographics, membrane shedding, and treatment were collected.

RESULTS

We identified 29 cases. Congenital ichthyosiform erythroderma and lamellar ichthyosis were the most common final diagnoses with 7 of 29 cases (24%) each; 3 patients were given the diagnosis of a syndromic ichthyosis. The classic nonsyndromic ichthyoses had higher average score results (7.33) than the syndromic ichthyoses (2.0) and other presentations (4.0), (P = .0097). Patients with major complications had higher, but nonsignificant, average severity scores (P = .64).

LIMITATIONS

The retrospective design and small number of patients with a syndromic ichthyosis are limitations.

CONCLUSIONS

Prospective studies are required to validate the proposed disease severity score.

摘要

背景

新生儿胶样膜可能是多种不同疾病的初始表现。目前缺乏将临床受累程度与潜在疾病和预后相关联的数据。

目的

我们试图通过疾病严重程度评分，确定胶样膜患者队列中预测最终结局和并发症的特征。

方法

这是一项在 31 年期间对三级医疗机构中患有胶样膜的新生儿进行的回顾性队列研究。我们设计并应用了 0 至 15 分的严重程度评分，并将结果与最终诊断和并发症相关联。收集了人口统计学、膜脱落和治疗的数据。

结果

我们确定了 29 例病例。先天性鱼鳞癣性红皮病和板层状鱼鳞癣是最常见的最终诊断，各有 7 例（24%）；3 例被诊断为综合征性鱼鳞癣。经典的非综合征性鱼鳞癣的平均评分结果（7.33）高于综合征性鱼鳞癣（2.0）和其他表现（4.0）（P =.0097）。有严重并发症的患者的平均严重程度评分较高，但无统计学意义（P =.64）。