Jaffar Huda, Shakir Zobia, Kumar Gaurav, Ali Iman Fatima
Dow University of Health Sciences Karachi Pakistan.
Skin Health Dis. 2022 Nov 25;3(1):e187. doi: 10.1002/ski2.187. eCollection 2023 Feb.
Ichthyosis vulgaris is an inherited, non-syndromic form of ichthyosis that presents with skin problems. Making up more than 95% cases of ichthyosis, ichthyosis vulgaris is caused by heterozygous loss-of-function mutation of the filaggrin gene, raising the fragility and permeability of the stratum corneum. It typically presents in infancy as xerosis, skin lesions, keratosis pilaris, palmoplantar hyper linearity, scaly dermatosis, and erythroderma, clearly identifiable by age 5. Although majority of patients have a normal lifespan, possible complications include a vitamin D deficiency and auditory problems due to scaling in the ears, besides a drop in quality of life due to dermatological changes. Urea-based creams with 10% urea, ceramides, and other ceramides are often the first line therapy in ichthyosis vulgaris. There is no known curative treatment for ichthyosis vulgaris, but lifelong treatment can alleviate the symptoms. Urea-based creams are highly therapeutic, whereas ammonium lactate 12% lotion with a physiological lipid-based repair cream can help with scaling and dryness. There is also evidence in favour of propylene glycol solutions. Risankizumab, an anti-interleukin-23 drug, and enhancement of natural moisturizing factors are also two highly promising solutions that require additional research. This review aims to provide updates on the manifestation, evaluation, and treatment of ichthyosis vulgaris.
寻常型鱼鳞病是一种遗传性、非综合征型鱼鳞病,会出现皮肤问题。寻常型鱼鳞病占鱼鳞病病例的95%以上,由丝聚合蛋白基因的杂合功能丧失突变引起,增加了角质层的脆性和通透性。它通常在婴儿期表现为皮肤干燥、皮肤病变、毛发角化病、掌跖纹明显、鳞屑性皮肤病和红皮病,在5岁时可明确诊断。虽然大多数患者寿命正常,但可能的并发症包括维生素D缺乏、由于耳部鳞屑导致的听力问题,以及由于皮肤变化导致的生活质量下降。含10%尿素、神经酰胺和其他神经酰胺的尿素乳膏通常是寻常型鱼鳞病的一线治疗方法。目前尚无治疗寻常型鱼鳞病的治愈方法,但终身治疗可缓解症状。尿素乳膏具有很高的治疗效果,而12%乳酸铵洗剂与生理性脂质修复乳膏可帮助缓解鳞屑和干燥。也有证据支持丙二醇溶液。抗白细胞介素-23药物司库奇尤单抗以及增强天然保湿因子也是两个非常有前景的解决方案,但需要更多研究。本综述旨在提供寻常型鱼鳞病的表现、评估和治疗的最新信息。