Department of General Medicine, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, 700-8558, Japan.
J Infect Chemother. 2022 Jul;28(7):978-981. doi: 10.1016/j.jiac.2022.02.021. Epub 2022 Mar 8.
Netherton's syndrome, a rare congenital disorder, is clinically characterized by chronic dermatologic disorders such as ichthyosiform erythroderma and ichthyosis linearis circumflexa. Curable treatment is yet to be established, and corticosteroid ointment is required to maintain good dermatological condition. Because of the permanent skin barrier impairment, patients with Netherton's syndrome are considered to be vulnerable to cutaneous infections. However, its clinical characteristics are yet to be elucidated due to the limited number of reported cases. Herein, we describe the clinical course of a patient who developed persistent methicillin-resistant Staphylococcus aureus (MRSA) bacteremia. A 19-year-old Japanese woman who had been diagnosed with Netherton's syndrome in her infancy and had been applying topical corticosteroid agents all over her body since her then, was referred to our hospital because of persistent MRSA bacteremia and secondary adrenal insufficiency. The patient was diagnosed with a central line-associated bloodstream infection and was appropriately treated with antibiotics and corticosteroid therapies. We assume that the damaged skin barrier due to the congenital dermatological disorder causes a disruption in the normal bacterial flora of the skin, leading to the invasion of harmful bacteria, such as S. aureus. In addition, internal (humoral immunodeficiency by decreased antibody against bacterial polysaccharide antigens) and external (prolonged and systemic use of corticosteroid ointment) factors bring about an immunodeficiency state in such patients. We highlight that in the absence of radical treatment, clinicians need to recognize that patients with Netherton's syndrome are vulnerable to bacterial infections owing to the mixture of immunosuppressive factors.
Netherton 综合征是一种罕见的先天性疾病，其临床特征为慢性皮肤疾病，如鱼鳞样红皮病和回旋状线状鱼鳞病。目前尚未确立有效的治疗方法，需要使用皮质类固醇软膏来维持良好的皮肤状况。由于永久性皮肤屏障损伤， Netherton 综合征患者容易发生皮肤感染。然而，由于报告的病例数量有限，其临床特征仍有待阐明。在此，我们描述了一位持续性耐甲氧西林金黄色葡萄球菌（MRSA）菌血症患者的临床病程。一位 19 岁的日本女性，在婴儿期被诊断为 Netherton 综合征，从那时起就全身涂抹外用皮质类固醇药物，因持续性 MRSA 菌血症和继发性肾上腺功能不全而被转至我院。该患者被诊断为中心静脉导管相关血流感染，给予适当的抗生素和皮质类固醇治疗。我们假设先天性皮肤病导致皮肤正常菌群破坏，使有害细菌（如金黄色葡萄球菌）入侵。此外，内在因素（针对细菌多糖抗原的抗体减少导致体液免疫缺陷）和外在因素（长期和全身性使用皮质类固醇软膏）使此类患者处于免疫缺陷状态。我们强调，在没有根治方法的情况下，临床医生需要认识到 Netherton 综合征患者由于存在多种免疫抑制因素，容易发生细菌感染。
