Norikawa Natsumi, Igari Shohei, Ishikawa Masato, Mori Tatsuhiko, Hiraiwa Tomoko, Kikuchi Nobuyuki, Hanami Yuka, Yamamoto Toshiyuki
Department of Dermatology, Fukushima Medical University, Fukushima, Japan.
Case Rep Dermatol. 2022 Sep 5;14(3):258-263. doi: 10.1159/000525382. eCollection 2022 Sep-Dec.
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.
落叶型天疱疮（PF）是红皮病的病因之一；然而，迄今为止，报道的病例相对较少。我们在此描述6例红皮病型PF。在所有6例病例中，PF是红皮病的直接病因，因为患者未接受任何医学治疗，既没有其他皮肤病，也未服用任何通常会引起红皮病的药物。6例中有5例血清IgE水平以及胸腺和活化调节趋化因子升高，而所有病例中可溶性白细胞介素-2受体和鳞状细胞癌相关抗原均显著增加，提示这些标志物是皮肤表面损伤的有力指标。所有患者均接受泼尼松龙（PSL）治疗，其中4例患者加用了PSL冲击治疗，4例患者加用了静脉注射免疫球蛋白。此外，除1例患者外，所有患者均为老年人，其中2例发生了疱疹样湿疹，并死亡，另外2例患者分别死于胃肠道出血和败血症。疱疹样湿疹是红皮病型PF的一种并发症，预后较差，因此在考虑诊断时需要谨慎。此外，老年人更易因PSL出现并发症，这可能导致死亡。不恰当的治疗和治疗延迟可能导致红皮病，所以早期诊断和治疗是必要的。
