Dermatology, Pediatrics and Pathology, Rutgers University New Jersey Medical School, New Jersey Medical School, Newark, New Jersey, USA.
Dermatol Ther. 2021 Jan;34(1):e14510. doi: 10.1111/dth.14510. Epub 2020 Nov 17.
Leiner's disease (LD) is a rare and serious syndrome of infantile erythroderma of severe and progressive generalized seborrheic-like dermatitis, recalcitrant diarrhea, malabsorption and wasting, and recurrent local and systemic infections. The purpose of this study is to provide an updated review on management with a summarized review of available peer-reviewed articles on LD. The mechanisms underlying this disease process remain unclear. The diagnosis includes demonstration of deficient opsonic activity along with the clinical tetrad of erythroderma, persistent gastrointestinal disturbance, superimposed bacterial or candidal infection, and marked wasting. An important correlation between LD and defective yeast and Staphylococcus aureus opsonization has been established. For the familial form of LD, an association of either complement three deficiency or complement five dysfunction has been made. LD should be distinguished from other types of infantile erythroderma, including Omenn syndrome. Treatment includes fluid and nutrition replacement, antibiotics to control infection, and fresh-frozen plasma therapy. The prognosis is unclear; it depends on treatment. LD is a life-threatening condition that requires prompt identification and hospitalization. Affected infants who receive vigorous treatment not only have the prospect of surviving, but also generally lead a normal life after infancy.
莱纳（Leiner）病是一种罕见且严重的综合征，表现为婴儿期红皮病，严重且进行性全身性脂溢性皮炎样皮疹，难治性腹泻，吸收不良和消瘦，以及反复发作的局部和全身感染。本研究的目的是提供一种关于莱纳病管理的最新综述，总结了关于莱纳病的现有同行评议文献。该病的发病机制尚不清楚。其诊断包括证明调理活性缺乏以及红皮病、持续胃肠道紊乱、继发细菌或假丝酵母菌感染和明显消瘦的四联征。莱纳病与酵母和金黄色葡萄球菌调理作用缺陷之间存在重要相关性。对于莱纳病的家族形式，已经确定了补体三缺乏或补体五功能障碍的关联。莱纳病应与其他类型的婴儿期红皮病（包括奥姆enn 综合征）相鉴别。治疗包括补液和营养补充、控制感染的抗生素以及新鲜冷冻血浆治疗。预后尚不清楚，取决于治疗情况。莱纳病是一种危及生命的疾病，需要及时识别和住院治疗。接受积极治疗的受影响婴儿不仅有生存的前景，而且在婴儿期后通常能够过上正常的生活。
