天疱疮(PF)是红皮病的原因之一;然而,到目前为止,报告的病例相对较少。我们在此描述了6例红皮症PF。在所有6个案例中,PF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病,也没有服用任何通常导致红皮病的药物。6例中有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在所有病例中均显著增加,表明这些标记是皮肤表面损伤的有力指标。所有患者均接受predonisolon(PSL)治疗,其中4例患者加用PSL脉冲,4例患者加用静脉注射免疫球蛋白。此外,除一名患者外,所有患者均为老年人,其中2例发生卡波西水痘型喷发,死了,还有另外两个病人,分别,死于消化道出血和败血症。卡波西静脉曲张型爆发是与预后不良相关的红皮PF的并发症。因此,在考虑诊断时需要谨慎。此外,老年人更容易因PSL而出现并发症,这可能会导致死亡。不适当的治疗和延迟治疗可能会导致红皮病,所以早期诊断和治疗是必要的。
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.