The Birmingham Epidermolysis Bullosa Severity score: development and validation
The Birmingham Epidermolysis Bullosa Severity score: development and validation
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DOI：
10.1111/j.1365-2133.2009.09041.x
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发表时间：
2009-05-01
影响因子：
10.3
通讯作者：
Davies, P.
中科院分区：
文献类型：
--
作者：
Moss, C.;Wong, A.;Davies, P.
客观严重度评分有助于临床护理和研究。然而，大疱性表皮病（EB）的罕见性和异质性使得评分困难，为了开发一个简单、有效、敏感和可靠的、涵盖所有年龄段EB所有亚型的严重程度评分，通过专家共识产生评分项目和权重，并根据内容效度和表面效度进行改进。对97例0-64岁患者进行了伯明翰EB严重程度评分（BEBS），共11项评分：皮肤受损面积、指甲、口腔、眼睛、喉和食管受累、手部瘢痕、皮肤癌、慢性伤口、脱发和营养不良。面积50分，其他10项各5分，满分100分。最低BEBS评分发生在Weber-Cockayne EB单纯型（中位数1.0;范围0.1-3.0; n = 12），全身性非Herlitz交界性EB评分最高（28.5; 5.0-62.0; n = 7），Hallopeau-Siemens隐性营养不良型EB（HS-RDEB）（22.9; 4.3-69.0; n = 23）和Herlitz交界性EB（H-JEB）（14.4; 2.5-49.3; n = 9），显性营养不良性EB的评分为中等（5.3; 0.5-15.9; n = 19）、Dowling-Meara EB单纯型（DM-EBS）（6.3; 2.8-22.5; n = 16）和非Hallopeau-Siemens隐性营养不良性EB（7.8，2.8-27.8; n = 11）。观察者内和观察者间的相关性很高。随着年龄的增长，H-JEB（r = 0.9，P = 0.001）和HS-RDEB（r = 0.73，P = 0.001）评分增加，DM-EBS（r =-0.62，P = 0.01）评分降低，与其他类型呈正相关，但无显著性。
Objective severity scores facilitate clinical care and research. However, the rarity and heterogeneity of epidermolysis bullosa (EB) make scoring difficult.To develop a severity score covering all subtypes of EB at all ages that is simple, valid, sensitive and reliable.Score items and weightings were generated by expert consensus, and refined for content and face validity. The Birmingham EB Severity (BEBS) score was tested on 97 patients aged 0-64 years.Eleven items were scored: area of damaged skin, involvement of nails, mouth, eyes, larynx and oesophagus, scarring of hands, skin cancer, chronic wounds, alopecia and nutritional compromise. Area was allocated 50 points, and the 10 other items 5 points each, giving a maximum score of 100. Lowest BEBS scores occurred in Weber-Cockayne EB simplex (median 1.0; range 0.1-3.0; n = 12), highest scores in generalized non-Herlitz junctional EB (28.5; 5.0-62.0; n = 7), Hallopeau-Siemens recessive dystrophic EB (HS-RDEB) (22.9; 4.3-69.0; n = 23) and Herlitz junctional EB (H-JEB) (14.4; 2.5-49.3; n = 9), and intermediate scores in dominant dystrophic EB (5.3; 0.5-15.9; n = 19), Dowling-Meara EB simplex (DM-EBS) (6.3; 2.8-22.5; n = 16) and non-Hallopeau-Siemens recessive dystrophic EB (7.8, 2.8-27.8; n = 11). Intra- and interobserver correlations were high. With age, scores increased for H-JEB (r = 0.9, P = 0.001) and HS-RDEB (r = 0.73, P = 0.001) and decreased for DM-EBS (r = -0.62, P = 0.01), with positive but nonsignificant correlations for the other types.The BEBS score appears valid and reproducible, gives appropriate scores for different subtypes, and reflects changes with age.