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一名患有特应性皮炎的患者发生落叶型天疱疮:病例报告

Pemphigus Foliaceus in a Patient With Pre-existing Atopic Dermatitis: A Case Report.

作者信息

AlSharhan Fahad, AlQusaimi Reem, AlAwadhi Doaa, AlRujaib Fawziah, Alebrahim Zainab, AlHashel Maryam, Alhasawi Afnan, Nasser Rawan, Alyousef Shireefa

机构信息

Dermatology, Abdulkareem AlSaeed Dermatology Center, Kuwait City, KWT.

Medicine and Surgery, Jaber AlAhmad Hospital, Kuwait City, KWT.

出版信息

Cureus. 2025 Jul 18;17(7):e88268. doi: 10.7759/cureus.88268. eCollection 2025 Jul.

DOI:10.7759/cureus.88268
PMID:40831828
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12358641/
Abstract

Pemphigus foliaceus (PF) is a rare autoimmune blistering skin disease characterized by superficial erosions and crusts due to autoantibodies targeting desmoglein 1. Diagnosis can be challenging, particularly when initial presentation mimics common dermatoses such as atopic dermatitis. We report the case of a 55-year-old Syrian female initially diagnosed with severe atopic dermatitis, who presented with widespread pruritic skin lesions unresponsive to corticosteroids. Clinical deterioration with new vesiculobullous lesions prompted re-evaluation. Histopathology and serologic testing confirmed PF. The patient achieved disease control with systemic corticosteroids and immunosuppressive therapy and remains in remission after two years of follow-up. This case underscores the diagnostic complexity of PF, especially in patients with overlapping or misleading dermatologic histories. A high index of suspicion, timely biopsy, and serological confirmation are essential for diagnosis. Long-term immunosuppressive therapy combined with corticosteroids proved effective in achieving sustained remission.

摘要

落叶型天疱疮(PF)是一种罕见的自身免疫性水疱性皮肤病,其特征是由于针对桥粒芯糖蛋白1的自身抗体导致表皮糜烂和结痂。诊断可能具有挑战性,尤其是当最初表现类似于特应性皮炎等常见皮肤病时。我们报告了一例55岁的叙利亚女性病例,最初被诊断为重度特应性皮炎,她出现了广泛的瘙痒性皮肤病变,对皮质类固醇治疗无反应。新的水疱大疱性病变导致临床病情恶化,促使重新评估。组织病理学和血清学检测确诊为PF。患者通过全身皮质类固醇和免疫抑制治疗实现了疾病控制,随访两年后仍处于缓解期。该病例强调了PF的诊断复杂性,尤其是在有重叠或误导性皮肤病史的患者中。高度的怀疑指数、及时活检和血清学确诊对于诊断至关重要。长期免疫抑制治疗联合皮质类固醇被证明对实现持续缓解有效。

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本文引用的文献

1
Pemphigus relapse: Mechanisms, risk factors, and agents associated with disease recurrence.天疱疮复发:与疾病复发相关的机制、危险因素及药物
J Dermatol. 2024 Dec;51(12):1533-1546. doi: 10.1111/1346-8138.17505. Epub 2024 Oct 26.
2
State-of-the-art diagnosis of autoimmune blistering diseases.自身免疫性水疱病的最新诊断方法。
Front Immunol. 2024 Jun 6;15:1363032. doi: 10.3389/fimmu.2024.1363032. eCollection 2024.
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Current biologics in treatment of pemphigus foliaceus: a systematic review.当前生物制剂治疗落叶型天疱疮:系统评价。
Front Immunol. 2023 Oct 12;14:1267668. doi: 10.3389/fimmu.2023.1267668. eCollection 2023.
4
Is transition between subtypes of pemphigus possible? A series of pemphigus vulgaris patients showing the transition to pemphigus foliaceus.各亚型天疱疮间是否可能相互转化?一组寻常型天疱疮患者转变为落叶型天疱疮。
An Bras Dermatol. 2023 Nov-Dec;98(6):787-792. doi: 10.1016/j.abd.2022.09.012. Epub 2023 Jun 23.
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An Updated Review of Pemphigus Diseases.天疱疮疾病的最新综述
Medicina (Kaunas). 2021 Oct 9;57(10):1080. doi: 10.3390/medicina57101080.
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Differentiating Pemphigus Foliaceus From Pemphigus Vulgaris in Clinical Practice.临床实践中鉴别落叶型天疱疮与寻常型天疱疮。
Cureus. 2021 Sep 11;13(9):e17889. doi: 10.7759/cureus.17889. eCollection 2021 Sep.
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Intraepithelial autoimmune blistering dermatoses: Clinical features and diagnosis.上皮内自身免疫性水疱性皮肤病:临床特征和诊断。
J Am Acad Dermatol. 2021 Jun;84(6):1507-1519. doi: 10.1016/j.jaad.2020.11.075. Epub 2021 Mar 5.
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Refractory pemphigus foliaceous treated with rituximab.利妥昔单抗治疗难治性落叶型天疱疮。
BMJ Case Rep. 2019 May 29;12(5):e229026. doi: 10.1136/bcr-2018-229026.
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Fogo selvagem: endemic pemphigus foliaceus.火激红斑:地方性落叶型天疱疮。
An Bras Dermatol. 2018 Sep-Oct;93(5):638-650. doi: 10.1590/abd1806-4841.20188235.
10
Pemphigus Vulgaris and Pemphigus Foliaceus: Differences in Epidemiology and Mortality.寻常型天疱疮和落叶型天疱疮:流行病学和死亡率的差异
Acta Derm Venereol. 2017 Oct 2;97(9):1095-1099. doi: 10.2340/00015555-2706.