Coexistence of Mycosis Fungoides and Photosensitive or Autoimmune Diseases. The Therapeutic Challenge: A retrospective Case Series from a Tertiary Referral Center.

IF 1.8 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL
Israel Medical Association Journal Pub Date : 2025-06-01
Meital Oren-Shabtai, Assi Levi, Daniel Mimouni, Hadas Prag-Naveh, Elena Didkovsky, Elisheva Pokroy-Shapira, Emmilia Hodak, Iris Amitay-Laish
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Abstract

Background: Mycosis fungoides (MF) combined with photosensitive/autoimmune diseases has been reported, yet there are limited data regarding the therapeutic considerations in these patients, specifically phototherapy, a mainstay skin-directed treatment (SDT), being a relative or complete contra-indication.

Objectives: To outline therapeutic considerations for patients with MF who had also been diagnosed with photosensitive/autoimmune diseases.

Methods: We conducted a retrospective analysis of patients with MF who were treated at our center between January 2008 and December 2024with photosensitive/autoimmune diseases, especially collagen vascular diseases (CVD) or autoimmune bullous diseases (AIBD).

Results: Eight patients were diagnosed with MF at a median age of 39 years. Seven had early-stage (4-IA, 3-IB) and one had Sézary syndrome. Six early-stage MF patients were diagnosed with lupus erythematosus (LE, 4) or AIBD (2) and were treated with SDT (topical corticosteroids/chlormethine gel), systemic retinoid or methotrexate. A patient with resistant early-stage MF and discoid LE was treated with electron beam and interferon. One patient who presented with variegate porphyria and localized MF was treated with electron beam. The patient with Sézary syndrome had inclusion body myositis. He was treated with low-dose total skin electron beam, methotrexate, extracorporeal photopheresis, and subsequently with romidepsin. After a median of 8 years, no stage progression of MF was observed. The Sézary syndrome patient achieved down-staging and was at stage IB. There was no aggravation of the co-morbidity in any of the patients.

Conclusions: Effective management of MF and associated photosensitive or autoimmune co-morbidities underscore the need for individualized treatment strategies in patients with these unique dual diagnoses.

蕈样真菌病与光敏性或自身免疫性疾病的共存。治疗的挑战:回顾性病例系列从三级转诊中心。
背景:真菌样真菌病(MF)合并光敏/自身免疫性疾病已被报道,但关于这些患者的治疗考虑的数据有限,特别是光疗,主要的皮肤定向治疗(SDT),是一个相对或完全的禁忌症。目的:概述诊断为光敏性/自身免疫性疾病的MF患者的治疗注意事项。方法:回顾性分析2008年1月至2024年12月在本中心治疗的光敏性/自身免疫性疾病,特别是胶原血管疾病(CVD)或自身免疫性大疱性疾病(AIBD)的MF患者。结果:8例患者被诊断为MF,中位年龄39岁。7例为早期(4-IA, 3-IB), 1例为ssamzary综合征。6例早期MF患者被诊断为红斑狼疮(LE, 4)或AIBD(2),并接受SDT(外用皮质类固醇/氯甲基凝胶)、系统性类视黄醇或甲氨蝶呤治疗。对1例早期耐药MF合并盘状LE患者采用电子束联合干扰素治疗。1例表现为多样卟啉症和局限性MF的患者采用电子束治疗。ssamzary综合征患者有包涵体肌炎。患者接受低剂量全皮肤电子束、甲氨蝶呤、体外光化学治疗,随后接受罗米地辛治疗。中位8年后,没有观察到MF的分期进展。ssamzary综合征患者达到了低分期,处于IB期。所有患者的合并症均未加重。结论:对MF和相关光敏或自身免疫性合并症的有效管理强调了对具有这些独特双重诊断的患者进行个体化治疗策略的必要性。
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来源期刊
Israel Medical Association Journal
Israel Medical Association Journal 医学-医学:内科
CiteScore
2.20
自引率
12.50%
发文量
54
审稿时长
3-8 weeks
期刊介绍: The Israel Medical Association Journal (IMAJ), representing medical sciences and medicine in Israel, is published in English by the Israel Medical Association. The Israel Medical Association Journal (IMAJ) was initiated in 1999.
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