文献简介

出版社:the American Academy of Dermatology, Inc.

作  者:Mohammad A. El-Darouti, MD, Marwa M. Fawzy, MD, Rehab A. Hegazy, MD, and Rania M. Abdel Hay, MD Cairo, Egypt

编  号:doi:10.1016/j.jaad.2012.02.030

关键字:digitiform extensions; follow-up; hypopigmented; mycosis fungoides; parapsoriasis en plaque; phototherapy.

年  份:2012   点击量:923

文献摘要

Background: In the past 7 years we have extensively studied an uncommon hypopigmented disorder that,apart from hypopigmentation, showed many common features with parapsoriasis en plaque (PSEP), both clinically and histopathologically.

研究背景:在过去的7年中,我们广泛研究了一种罕见的色素减退型疾病,除了色素减退,在临床和组织病理学上,这种疾病还表现出许多与斑块状副银屑病(PSEP)共同的特征。

Objective: We sought to verify whether this disorder should be considered a hypopigmented variant of PSEP and thus be referred to as hypopigmented PSEP.

研究目的:我们试图验证这种疾病是否应被视为PSEP的色素减退型变体,因而被称为色素减退型PSEP。

Methods: A total of 34 patients presenting with this peculiar hypopigmented disorder were included(2003-2010). Patients were subjected to a predesigned algorithm excluding all possible differential diagnoses of hypopigmented lesions.

研究方法:共有34例出现这种奇特色素减退型疾病的患者被纳入本项研究。患者受到了一个预先设计的算法,该算法不包括所有可能的色素减退型疾病的鉴别诊断。

Results: Our findings indicated that this disorder can be diagnosed as hypopigmented PSEP. These findings included:

(1) exclusion of all other disorders causing similar hypopigmented lesions;

(2) shape and size of the lesions being very similar to those of classic small PSEP (small-plaque parapsoriasis [SPP]);

(3) similar distribution of the lesions (trunk, proximal upper and lower limbs) to the classic PSEP; (4) digitiform extensions of most the lesions (70.5% of our patients) as in SPP;

(5) absence of itching as in PSEP (SPP type);

(6) good response to narrowband ultraviolet B in 76.4% of the patients (n = 26); and

(7) during follow-up 5 patients (14.7%) converted into hypopigmentd mycosis fungoides.

研究结果:我们的研究结果表明这种疾病可以诊断为色素减退PSEP。该项发现包括:

1.  排除引起类似的色素减退型病变的所有其他疾病。

2.  皮损的形状和大小类似于经典的小斑块型副银屑病(SPP)。

3.  皮损的分布(躯干、四肢近端)类似经典的斑块型副银屑病。

4.  在SPP中大多数皮损呈指状型扩展(70.5%的患者)。

5.  在PSEP中无瘙痒出现(SPP型)。

6.  76.4%的患者在窄带紫外线B的治疗中有良好的反应。

7.  随访期间有5例患者(14.7%)转换成了色素减退型蕈样肉芽肿。

Limitations: A limitation in our study is that we did not perform clonal T-cell receptor gene rearrangement because of limited resources.

研究局限:由于资源有限,我们研究的局限性在于没有进行克隆T细胞受体基因的重排。

Conclusion: Based on our findings we believe that this hypopigmented disorder is a well-defined new variant of the PSEP family that shows, apart from the hypopigmentation, all the features of PSEP, particularly the SPP variant,  and accordingly could be referred to as hypopigmented PSEP.

研究结论:根据研究结果,我们相信这种色素减退型疾病是PSEP家族中一个定义明确的新变种,除了色素减退,它还具有PSEP的所有特点,尤其是SPP变体,因此都相应的可以被称为色素减退型PSEP。