Biondo Michela Ileen, Fiorentino Chiara, Persechino Severino, Tammaro Antonella, Koverech Angela, Bartolazzi Armando, Raffa Salvatore, Canzoni Marco, Picchianti-Diamanti Andrea, Di Rosa Roberta, Di Zenzo Giovanni, Scala Enrico, Meneguzzi Giorgia, Ferlito Claudia, Markovic Milica, Caporuscio Sara, Sorgi Maria Laura, Salemi Simonetta, Laganà Bruno
Dipartimento di Medicina Clinica e Molecolare, Sapienza, Università di Roma, AOU S. Andrea, 00189 Roma, Italy.
UOD di Dermatologia, Sapienza, Università di Roma, AOU S. Andrea, 00189 Roma, Italy.
Microorganisms. 2021 Jun 7;9(6):1235. doi: 10.3390/microorganisms9061235.
Bullous pemphigoid (BP) is an autoimmune blistering skin disease, mainly observed in the elderly. Infections have been suggested as possible disease triggers. However, infections may even heavily influence the disease clinical course and mortality. A 75-year-old woman was admitted to hospital for severe erythematosus blistering disease, accompanied by hyper-eosinophilia and hyper-IgE. The culture of bullous fluid was positive for , the blood culture was positive for , and the urine culture was positive for and Moreover, circulating anti-BP180 IgG was present and the histopathological/ultrastructural examination of a lesional skin biopsy was compatible with BP. High eosinophil levels (up to 3170/µL) were found throughout the clinical course, while values below 1000/µL were associated with clinical improvement. The total IgE was 1273 IU/mL, and specific anti-G/V-penicillin/ampicillin IgE antibodies were positive. The patient had a complete clinical recovery in two months with methyl-prednisolone (40 then 20 mg/day) and low-dose azathioprine (50 mg/day) as a steroid-sparing agent. The steroid treatment was tapered until interruption during a one-year period and intravenous immunoglobulins have been administered for three years in order for azathioprine to also be interrupted. The patient stopped any treatment five years ago and, in this period, has always been in good health. In this case, the contemporaneous onset of different bacterial infections and BP is suggestive of bacterial infections acting as BP trigger(s), with allergic and autoimmune pathways contributing to the disease pathogenesis.
大疱性类天疱疮(BP)是一种自身免疫性水疱性皮肤病,主要见于老年人。感染被认为是可能的疾病触发因素。然而,感染甚至可能严重影响疾病的临床病程和死亡率。一名75岁女性因严重红斑性水疱病入院,伴有嗜酸性粒细胞增多和高IgE血症。水疱液培养对 呈阳性,血培养对 呈阳性,尿培养对 和 呈阳性。此外,存在循环抗BP180 IgG,病变皮肤活检的组织病理学/超微结构检查与BP相符。在整个临床病程中发现嗜酸性粒细胞水平较高(高达3170/µL),而低于1000/µL的值与临床改善相关。总IgE为1273 IU/mL,特异性抗G/V-青霉素/氨苄青霉素IgE抗体呈阳性。患者在使用甲泼尼龙(40mg/天,后减至20mg/天)和低剂量硫唑嘌呤(50mg/天)作为激素替代药物治疗两个月后临床完全康复。激素治疗在一年内逐渐减量直至停药,静脉注射免疫球蛋白已使用三年以便也停用硫唑嘌呤。患者五年前停止任何治疗,在此期间一直健康状况良好。在该病例中,不同细菌感染与BP同时发生提示细菌感染作为BP的触发因素,过敏和自身免疫途径参与了疾病的发病机制。