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20例韩国毛囊性蕈样肉芽肿病例:临床和组织病理学特征及对紫外线A-1和/或光动力疗法的反应

Folliculotropic Mycosis Fungoides in 20 Korean Cases: Clinical and Histopathologic Features and Response to Ultraviolet A-1 and/or Photodynamic Therapy.

作者信息

Jang Min Soo, Jang Ji Yun, Park Jong Bin, Kang Dong Young, Lee Jin Woo, Lee Taek Geun, Hwangbo Hyun, Suh Kee Suck

机构信息

Department of Dermatology, Kosin University College of Medicine, Busan, Korea.

Department of Dermatology, Maryknoll Medical Center, Busan, Korea.

出版信息

Ann Dermatol. 2018 Apr;30(2):192-201. doi: 10.5021/ad.2018.30.2.192. Epub 2018 Feb 21.

Abstract

BACKGROUND

Folliculotropic mycosis fungoides (FMF) is a variant of mycosis fungoides (MF) that is characterized clinically by variable types of skin eruptions, including plaques, acneiform lesions, and alopecic patches. Histopathologically, FMF is characterized by folliculotropic infiltrates.

OBJECTIVE

This study was conducted to scrutinize the clinical and histopathologic features of FMF in Koreans and the responses to phototherapy.

METHODS

Twenty Koreans diagnosed with MF who had histopathologic evidence of folliculotropism were enrolled.

RESULTS

Eighteen patients had head-and-neck-region infiltration, while five had solitary lesion. In all patients, the atypical lymphocytic infiltrate had a perifollicular distribution. Twelve patients were treated with ultraviolet A (UVA)-1. Eleven of these 12 patients with early-stage FMF experienced >80% improvement (8: complete remission; 3: partial remission). Four patients, including 2 who relapsed after UVA-1, were treated with photodynamic therapy (PDT), reaching complete remission after PDT.

CONCLUSION

As FMF has variable clinical presentations, skin biopsy is required to confirm the diagnosis. And both UVA-1 and methyl aminolevulinate-PDT are clinically effective in treatment of early-stage FMF.

摘要

背景

毛囊性蕈样肉芽肿(FMF)是蕈样肉芽肿(MF)的一种变体,其临床特征为多种类型的皮肤疹,包括斑块、痤疮样损害和秃发性斑片。组织病理学上,FMF的特征为毛囊性浸润。

目的

本研究旨在仔细观察韩国人FMF的临床和组织病理学特征以及对光疗的反应。

方法

纳入20例经组织病理学证实有毛囊性倾向的韩国MF患者。

结果

18例患者有头颈部浸润,5例有孤立性损害。所有患者中,非典型淋巴细胞浸润呈毛囊周围分布。12例患者接受了紫外线A(UVA)-1治疗。这12例早期FMF患者中有11例改善>80%(8例:完全缓解;3例:部分缓解)。4例患者,包括2例UVA-1治疗后复发的患者,接受了光动力疗法(PDT),PDT后达到完全缓解。

结论

由于FMF临床表现多样,需要进行皮肤活检以确诊。UVA-1和甲基氨基酮戊酸-PDT在早期FMF的治疗中均具有临床疗效。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9fe4/5839891/54f90480a74a/ad-30-192-g001.jpg

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