Martínez-Calvo Julián, Correa-Jimenez Oscar, Alfaro-Murillo Alberto
Internal Medicine Department, Hospital San Juan de Dios, San José, CRI.
Pulmonology and Immunology in Pediatrics Research Group, School of Medicine, Universidad Nacional de Colombia, Bogotá, COL.
Cureus. 2024 Oct 18;16(10):e71799. doi: 10.7759/cureus.71799. eCollection 2024 Oct.
Gestational pemphigoid is a rare autoimmune skin condition specific to pregnancy and the postpartum period, with a variable course. There are currently no standardized guidelines referring to evidence-based therapeutic strategies. Intravenous immunoglobulin (IVIG) has recently emerged as a safe and effective steroid-sparing option as a second-line treatment for cases refractory to conventional steroid therapy and for managing relapses. We present the case of a 36-year-old primigravida patient diagnosed with gestational pemphigoid who initially had a difficult clinical course despite treatment with high-dose oral steroids. A first cycle of IVIG combined with steroids (with a gradual dose tapering regimen) was administered, showing initial clinical improvement. However, the patient experienced a relapse that required a new increase in steroid dose and interruption of pregnancy due to fetal growth restriction. Given persistent disease activity at the first outpatient follow-up, a combination of oral steroids with a gradual dose tapering regimen, along with a new cycle of IV immunoglobulin and azathioprine, was initiated, leading to complete resolution by the 19th week of outpatient follow-up. No adverse effects associated with IVIG were reported during the course of follow-up.
妊娠类天疱疮是一种罕见的自身免疫性皮肤病，特定于妊娠期和产后时期，病程多变。目前尚无基于证据的治疗策略的标准化指南。静脉注射免疫球蛋白（IVIG）最近已成为一种安全有效的辅助激素治疗方法，可作为常规激素治疗无效病例的二线治疗以及用于控制复发。我们报告一例36岁初产妇，诊断为妊娠类天疱疮，尽管接受了高剂量口服激素治疗，最初临床过程仍很艰难。给予了第一个周期的IVIG联合激素治疗（采用逐渐减量方案），显示出初步的临床改善。然而，患者复发，需要再次增加激素剂量，并因胎儿生长受限而中断妊娠。鉴于首次门诊随访时疾病持续活动，开始采用逐渐减量方案的口服激素联合新周期的静脉注射免疫球蛋白和硫唑嘌呤治疗，在门诊随访第19周时病情完全缓解。随访过程中未报告与IVIG相关的不良反应。
