Stevens Johnson Syndrome: Past, Present, and Future Directions Gynecologic Manifestations and Management in SJS/TEN.
Stevens Johnson Syndrome: Past, Present, and Future Directions Gynecologic Manifestations and Management in SJS/TEN.
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DOI：
10.3389/fmed.2022.874445
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发表时间：
2022
影响因子：
3.9
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中科院分区：
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史蒂文斯约翰逊综合征（SJS）和中毒性表皮坏死松解症（TEN）是严重的皮肤粘膜过敏性疾病，其特征是突然发生表皮坏死。SJS/TEN的急性表现通常包括外阴阴道糜烂、溃疡、阴道分泌物、出血、阴道疼痛、排尿困难和尿潴留。如果不治疗，这可能导致并发症，如外阴阴道粘连、阴道狭窄或干燥、疼痛、性交困难、出血和腺病。即使有适当的治疗，也会有持久的影响，包括阴道检查困难和心理困扰。早期识别和治疗外阴阴道受累是预防严重后遗症的关键。尽管SJS/TEN累及泌尿生殖系统具有潜在的破坏性后果，但涉及外阴和阴道粘膜皮肤表面的文献不一致，治疗和随访方案也不完善。外阴阴道受累的治疗很大程度上依赖于专家意见，并且很少有关于建议的管理效果的数据。本综述的目的是确定建立一种临床途径是否能增加外阴阴道SJS/TEN的治疗，并优化我们的标准化方案，以预防泌尿生殖系统后遗症。我们对2008年至2021年在华盛顿大学港景医学中心的SJS/TEN女性患者进行了回顾性图表回顾。从电子病历中收集人口统计和临床数据，包括妇科咨询、检查结果、治疗方案和门诊随访。我们比较了2017年实施临床护理路径前后的数据。我们回顾了2008年至2021年间可能患有SJS/TEN的女性的88张图表。在这88张图表中，77张被发现有明确的活检证实诊断为SJS/TEN。共有42例患者发现外阴阴道受累（55%），其中43%的病例咨询了妇科。50%的外阴-阴道受累患者（n = 21）建议使用阴道扩张器和类固醇软膏治疗，34%的外阴-阴道受累患者未接受治疗。2008年至2017年5月（方案前），我们发现55例SJS/TEN患者。55%的患者（n = 29）有外阴阴道受累（n = 26外阴，n = 21阴道）。只有26% （n = 14）的患者咨询了妇科。在21名阴道受累的女性中，只有38% （n = 8）推荐使用类固醇软膏的扩张器/阴道模具。在26名外阴累及的女性中，31% （n = 8）没有外阴治疗建议，其余69%有一些记录，从仅放置纱布（19%）到外用利多卡因、屏障霜、抗生素或抗真菌霜/软膏、润滑剂或外用类固醇软膏（50%）。38% （n = 9）的经期女性推荐使用月经抑制。只有4%的患者开了抗真菌药物。在2017年实施SJS/TEN临床治疗途径后，确定了22名女性SJS/TEN患者。72% （n = 16）有外阴阴道受累（n = 16外阴，n = 9阴道）。86% （n = 19）的患者进行了妇科咨询。在协议实施后，我们确定了几项改进。妇科咨询总体上从方案前的26%增加到方案后的86%。对于外阴阴道受累的患者，会诊完成率为93%，而方案前为50%。值得注意的是，外阴阴道病变的发现从53%增加到72%。扩张器与局部类固醇软膏的使用一直被推荐，作为抗真菌和月经抑制的使用。制定了女性SJS/TEN患者的治疗方案，增加了妇科会诊的一致性以及外阴阴道SJS/TEN的记录和治疗。我们发现有必要改善出院后的临床随访，这可以安排为多学科随访，是评估长期结果（疼痛、性活动等）的一个很好的选择。至于未来的方向，我们正在评估有关生活质量和性功能的长期数据。需要确定急性环境下治疗对慢性后遗症发展的影响，以及对阴道干燥、疼痛、性交困难等长期后遗症的管理。局部雌激素与阴道激光的作用有待进一步探讨。盆底物理治疗可能在康复中发挥重要作用，但尚未研究。
Stevens Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are severe mucocutaneous hypersensitivity disorders characterized by sudden onset epidermal necrosis. Acute manifestations of SJS/TEN often include vulvovaginal erosions, ulcerations, vaginal discharge, bleeding, vaginal pain, dysuria, and urinary retention. If not treated, this can lead to complications such as vulvovaginal adhesions, vaginal stenosis or dryness, pain, dyspareunia, bleeding, and adenosis. Even with adequate treatment, there are lasting impacts including difficulty with vaginal exams and psychological distress. Early recognition and treatment of vulvovaginal involvement are crucial to preventing severe sequelae. Despite the potentially devastating consequences of genitourinary involvement of SJS/TEN, involvement of the mucocutaneous surfaces of the vulva and vagina is inconsistently documented, and protocols for treatment and follow-up are not well-established. The treatment of vulvovaginal involvement relies largely on expert opinion, and there is little data on the efficacy of suggested management. The goal of this review was to identify whether establishing a clinical pathway increased treatment of vulvovaginal SJS/TEN and to optimize our standardized protocol to prevent genitourinary sequelae. We conducted a retrospective chart review of female patients with SJS/TEN at Harborview Medical Center, University of Washington from 2008 to 2021. Demographic and clinical data including gynecologic consultation, exam findings, treatment regimens, and outpatient follow-up were collected from the electronic medical record. We compared data before and after implementation of a clinical care pathway in 2017. We reviewed a total of 88 charts of women with possible SJS/TEN between 2008 and 2021. Of these 88 charts, 77 were found to have clear biopsy proven diagnosis of SJS/TEN. A total of 42 patients were found to have vulvovaginal involvement (55%) and gynecology was consulted in 43% of cases. 50% of patients (n = 21) with vulvovaginal involvement were recommended treatment with vaginal dilators and steroid ointment and 34% of patients with genital involvement received no treatment. Between 2008 and May of 2017 (pre-protocol), we found 55 patients with SJS/TEN. 55% of patients (n = 29) had vulvovaginal involvement (n = 26 vulvar, n = 21 vaginal). Gynecology was only consulted in 26% (n = 14) of patients. Of the 21 females with vaginal involvement, only 38% (n = 8) had dilators/vaginal molds with steroid ointment recommended. Of the 26 females with vulvar involvement, 31% (n = 8) had no vulvar treatment recommendations with the remaining 69% having some documentation that ranged from gauze placement only (19%) to topical lidocaine, barrier cream, antibiotic or antifungal cream/ ointment, lubricant, or topical steroid ointment (50%). Menstrual suppression was recommended in 38% (n = 9) of menstruating females. An antifungal medication was only prescribed in 4% of patients. Following implementation of the clinical pathway for the treatment of SJS/TEN in 2017, 22 females with SJS/TEN were identified. 72% (n = 16) had documented vulvovaginal involvement (n = 16 vulvar, n = 9 vaginal). Gynecology consultations took place in 86% (n = 19) of patients. We identified several improvements after implementation of the protocol. Gynecology consults overall increased from 26% pre-, to 86% post-protocol. For patients with vulvovaginal involvement, consultations were completed in 93% compared to 50% prior to protocol. Of note, the finding of vulvovaginal lesions increased from 53 to 72%. Dilator use with topical steroid ointment was consistently recommended, as was antifungal use and menstrual suppression. Having a protocol in place for treatment of female patients with SJS/TEN increased the consistency of Gynecologic consultation and the documentation and treatment of vulvovaginal SJS/TEN. We identified the need to improve clinical follow-up after discharge from the hospital, which could be arranged as multidisciplinary visits and would be a good option to assess long-term outcomes (pain, sexual activity, etc.). With regards to future directions, we are in the process of assessing long-term data on quality of life and sexual functioning. The impact of treatment in the acute setting on the development of chronic sequelae needs to be established, as does the management of long-term sequelae like vaginal dryness, pain, dyspareunia. The role of local estrogen and vaginal laser still needs to be explored. Pelvic floor physical therapy might play a significant role in rehabilitation and has yet to be studied.
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影响因子：
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作者：
Magone MT;Maiberger M;Clayton J;Pasieka H
通讯作者：
Pasieka H
影响因子：
7.2
作者：
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通讯作者：
Paniel, BJ
影响因子：
2.7
作者：
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通讯作者：
Chodosh, James
影响因子：
13.8
作者：
O'Brien, Kathleen F.;Bradley, Sarah E.;Pasieka, Helena B.
通讯作者：
Pasieka, Helena B.