Folliculotropic Mycosis Fungoides - A Case Report

Ivana Ilijin, Z. Mijuskovic, D. Brasanac, L. Kandolf Sekulovič
{"title":"Folliculotropic Mycosis Fungoides - A Case Report","authors":"Ivana Ilijin, Z. Mijuskovic, D. Brasanac, L. Kandolf Sekulovič","doi":"10.1515/sjdv-2016-0019","DOIUrl":null,"url":null,"abstract":"Abstract Mycosis fungoides (MF) belongs to a group of primary cutaneous T-cell lymphomas, with characteristic small- to medium-sized neoplastic T-lymphocytes with hyperchromatic and cerebriform nuclei. Folliculotropic mycosis fungoides (FMF) represents a variant of mycosis fungoides, which is histologically characterized by folliculotropic T-cell infiltrates, with or without mucinous degeneration of the hair follicles. Clinical features of FMF are characterized by appearance of grouped follicular papules, acneiform lesions, indurated plaques, sometimes tumors, which usually involve the head and neck region. The diagnosis is based on clinical presentation, histopathological and immunohistochemical (IHC) findings of skin biopsy specimens. The treatment of FMF, and generally MF, should be stage-adapted. Case report: We present a case of a 33-year-old male with an eight-month history of erythematous papules on his forehead accompanied by intense pruritus. Histopathological findings showed folliculotropic and perivascular lymphocytic infiltrates. An increased CD4/CD8 ratio of interfollicular lymphocytes with accumulation of Langerhans cell confirmed the diagnosis of FMF. Our patient was diagnosed with an early stage - IA, and P-UVA phototherapy was recommended due to ineffectiveness of prescribed topical corticosteroids that had shortterm effects. Conclusion: Folliculotropic mycosis fungoides represents a diagnostic challenge due to the great diversity of clinical manifestations. We presented a rare case of folliculotropic mycosis fungoides in a young adult, who presented with erythematous papules, accompanied by intense pruritus on the forehead, which lasted for several months. Histopathological and IHC analysis confirmed the diagnosis of folliculotropic mycosis fungoides stage IA. Due to an inadequate response to a topical corticosteroid, P-UVA phototherapy was administered, as well as close follow-up, essential for timely treatment of this frequently therapy-resistant disease.","PeriodicalId":30659,"journal":{"name":"Serbian Journal of Dermatology and Venereology","volume":"8 1","pages":"213 - 220"},"PeriodicalIF":0.0000,"publicationDate":"2016-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Serbian Journal of Dermatology and Venereology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1515/sjdv-2016-0019","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0

Abstract

Abstract Mycosis fungoides (MF) belongs to a group of primary cutaneous T-cell lymphomas, with characteristic small- to medium-sized neoplastic T-lymphocytes with hyperchromatic and cerebriform nuclei. Folliculotropic mycosis fungoides (FMF) represents a variant of mycosis fungoides, which is histologically characterized by folliculotropic T-cell infiltrates, with or without mucinous degeneration of the hair follicles. Clinical features of FMF are characterized by appearance of grouped follicular papules, acneiform lesions, indurated plaques, sometimes tumors, which usually involve the head and neck region. The diagnosis is based on clinical presentation, histopathological and immunohistochemical (IHC) findings of skin biopsy specimens. The treatment of FMF, and generally MF, should be stage-adapted. Case report: We present a case of a 33-year-old male with an eight-month history of erythematous papules on his forehead accompanied by intense pruritus. Histopathological findings showed folliculotropic and perivascular lymphocytic infiltrates. An increased CD4/CD8 ratio of interfollicular lymphocytes with accumulation of Langerhans cell confirmed the diagnosis of FMF. Our patient was diagnosed with an early stage - IA, and P-UVA phototherapy was recommended due to ineffectiveness of prescribed topical corticosteroids that had shortterm effects. Conclusion: Folliculotropic mycosis fungoides represents a diagnostic challenge due to the great diversity of clinical manifestations. We presented a rare case of folliculotropic mycosis fungoides in a young adult, who presented with erythematous papules, accompanied by intense pruritus on the forehead, which lasted for several months. Histopathological and IHC analysis confirmed the diagnosis of folliculotropic mycosis fungoides stage IA. Due to an inadequate response to a topical corticosteroid, P-UVA phototherapy was administered, as well as close follow-up, essential for timely treatment of this frequently therapy-resistant disease.
嗜滤泡性蕈样真菌病1例报告
蕈样真菌病(Mycosis fungoides, MF)属于一组原发性皮肤t细胞淋巴瘤,特征性为小至中型肿瘤t淋巴细胞,核深染,呈脑状。嗜毛囊性蕈样真菌病(FMF)是蕈样真菌病的一种变体,其组织学特征是嗜毛囊性t细胞浸润,伴或不伴毛囊粘液变性。FMF的临床特征表现为成组的滤泡丘疹、痤疮样病变、硬化斑块,有时肿瘤,通常累及头颈部。诊断是基于临床表现,组织病理学和免疫组织化学(IHC)的皮肤活检标本的结果。FMF的治疗,以及一般的MF,应该根据阶段进行调整。病例报告:我们提出的情况下,一个33岁的男性与8个月的历史,红斑丘疹在他的前额,并伴有强烈的瘙痒。组织病理学表现为卵泡增生和血管周围淋巴细胞浸润。滤泡间淋巴细胞CD4/CD8比值升高,朗格汉斯细胞积聚,证实FMF的诊断。我们的患者被诊断为早期IA,由于处方局部皮质类固醇具有短期效果无效,因此建议使用P-UVA光疗。结论:嗜滤泡性蕈样真菌病临床表现多样,诊断难度大。我们提出了一个罕见的病例嗜滤泡性蕈样真菌病在一个年轻的成年人,谁提出了红斑丘疹,并伴有强烈的瘙痒在额头,这持续了几个月。组织病理学和免疫组化分析证实了嗜滤泡性蕈样真菌病IA期的诊断。由于对局部皮质类固醇的反应不足,因此给予P-UVA光疗以及密切随访,这对于及时治疗这种经常具有治疗抗性的疾病至关重要。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
求助全文
约1分钟内获得全文 求助全文
来源期刊
自引率
0.00%
发文量
0
期刊介绍: Serbian Journal of Dermatology and Venereology is a journal of the Serbian Association of Dermatologists and Venereologists. The journal is published in English, quarterly and intended to provide rapid publication of papers in the field of dermatology and venereology. Manuscripts are welcome from all countries in the following categories: editorials, original studies, review articles, professional articles, case reports, and history of medicine.
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:604180095
Book学术官方微信