Rheault Sylvie
Centre de recherche de l'Institut universitaire de gériatrie de Montréal/Université de Montréal, Montreal, QC, Canada.
SAGE Open Med Case Rep. 2021 Nov 18;9:2050313X211059295. doi: 10.1177/2050313X211059295. eCollection 2021.
Incontinentia pigmenti is a rare genetic disease affecting the skin, microvasculature, and central nervous system, in which a hyperactive inflammatory response is observed. Due to the inflammatory phase of COVID-19 and associated cytokine storm, infection with SARS-CoV-2 in individuals with incontinentia pigmenti is a concern. Furthermore, type I interferon autoantibodies are found in life-threatening COVID-19 pneumonia and in 25% of individuals with incontinentia pigmenti. The present case report describes a 31-year-old Caucasian woman with incontinentia pigmenti and severe COVID-19. She was hospitalized for oxygen therapy, intravenous antibiotics, and corticosteroids. Eight months later, she is still symptomatic. To our knowledge, she is the first reported case of long COVID in incontinentia pigmenti. Increased autoimmunity may be implicated in both incontinentia pigmenti and long COVID. Pending evidence-based guidelines, COVID-protective measures including vaccination should be recommended to all patients with incontinentia pigmenti. Specific interferon therapy may be considered along with usual COVID treatment.
色素失禁症是一种罕见的遗传性疾病，会影响皮肤、微血管和中枢神经系统，其中会观察到过度活跃的炎症反应。由于新冠病毒感染（COVID-19）的炎症期及相关的细胞因子风暴，色素失禁症患者感染严重急性呼吸综合征冠状病毒2（SARS-CoV-2）是一个值得关注的问题。此外，在危及生命的COVID-19肺炎患者以及25%的色素失禁症患者中发现了I型干扰素自身抗体。本病例报告描述了一名31岁患有色素失禁症且感染了重症COVID-19的白种女性。她因氧疗、静脉注射抗生素和使用皮质类固醇而住院。八个月后，她仍有症状。据我们所知，她是首例报告的色素失禁症患者出现长期新冠症状的病例。自身免疫性增加可能与色素失禁症和长期新冠症状都有关。在有循证指南之前，应向所有色素失禁症患者推荐包括接种疫苗在内的新冠防护措施。在进行常规的COVID治疗时可考虑使用特定的干扰素疗法。
