红皮病性天疱疮6例报告。

IF 0.9 Q4 DERMATOLOGY
Natsumi Norikawa, Shohei Igari, Masato Ishikawa, Tatsuhiko Mori, Tomoko Hiraiwa, Nobuyuki Kikuchi, Yuka Hanami, Toshiyuki Yamamoto
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引用次数: 1

摘要

天疱疮(Pemphigus foliaceus, PF)是红皮病的病因之一;然而,迄今为止,报告的病例相对较少。我们在此描述了6例红皮病性PF,在所有6例中,PF都是红皮病的直接原因,因为患者没有接受任何药物治疗,也没有任何其他皮肤疾病,也没有服用任何通常引起红皮病的药物。6例患者中有5例血清IgE、胸腺及活化调节趋化因子水平升高,而可溶性白细胞介素-2受体和鳞状细胞癌相关抗原均显著升高,提示这些标志物是皮肤表面损伤的有力指标。所有患者均给予强的松隆(PSL)治疗,其中4例患者加脉冲PSL, 4例患者加静脉免疫球蛋白。此外,除1例患者外,其余患者均为老年人,其中2例发生卡波西氏静脉曲张疹死亡,另有2例分别死于胃肠道出血和败血症。Kaposi的静脉曲张状喷发是红皮病性PF的并发症,预后差,因此在考虑诊断时必须谨慎。此外,老年人更容易因PSL而出现并发症,这可能导致死亡。治疗不当和治疗延误可能导致红皮病,因此早期诊断和治疗是必要的。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Six Cases of Erythrodermic Pemphigus Foliaceus: A Case Report.

Six Cases of Erythrodermic Pemphigus Foliaceus: A Case Report.

Pemphigus foliaceus (PF) is one of the causes of erythroderma; however, to date, there have been relatively few reported cases. We herein describe 6 cases of erythrodermic PF. In all 6 cases, PF was a direct cause of erythroderma because the patients had not undergone any medical treatments and neither had any other skin diseases nor were taking any drugs that typically cause erythroderma. Serum levels of IgE and thymus and activation-regulated chemokine were elevated in 5 of the 6 cases, whereas soluble interleukin-2 receptor and squamous cell carcinoma-related antigen were markedly increased in all cases, suggesting that those markers are strong indicators of skin surface damage. All patients were treated with predonisolon (PSL), of which PSL pulse was added in 4 patients and intravenous immunoglobulin was added in 4 patients. Furthermore, all patients except for one were older adults, among whom 2 cases developed Kaposi's varicelliform eruption, and died, and another 2 patients, respectively, died of gastrointestinal bleeding and sepsis. Kaposi's varicelliform eruption is a complication of erythrodermic PF associated with poor prognosis, and thus caution is necessary when considering the diagnosis. Furthermore, elderly people are more likely to have complications due to PSL, which may result in death. Inappropriate treatment and delay in treatment may cause erythroderma, so early diagnosis and treatment are necessary.

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来源期刊
CiteScore
1.60
自引率
0.00%
发文量
57
审稿时长
9 weeks
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