PNS Shifa Hospital, Karachi, Pakistan.
Department of Dermatology, PNS Shifa Hospital, Karachi, Pakistan.
J Pak Med Assoc. 2024 Sep;74(9):1687-1689. doi: 10.47391/JPMA.9785.
Erythrokeratodermia variabilis (EKV) is a rare inherited genodermatosis characterised by migratory and erythematous patches changing over the course of hours to days and fixed keratotic plaques. The disease begins mostly at birth or within the first year of life; it very rarely starts after childhood. We present here a sporadic case with adult onset EKV that was aggravated by pregnancy with spontaneous resolution, and later on, after crash diet more persistent patches and plaques appeared with no spontaneous resolution. The patient showed excellent response to systemic retinoids.
红细胞角化病(EKV)是一种罕见的遗传性皮肤病,其特征为迁移性和红斑性斑块在数小时至数天内变化,并形成固定的角化斑块。这种疾病大多在出生时或出生后的第一年开始发病;极少数情况下会在儿童期以后发病。我们在此介绍一例散发性成人发病 EKV 病例,该病例在妊娠期间加重,随后自发缓解,之后在节食后更持久的斑块和斑块出现,且无自发缓解。患者对系统类维生素 A 治疗反应良好。