文章基本信息

标题：Sporadic Progressive Symmetric Erythrokeratoderma: Classical Presentation of a rare Condition
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作者：Kompal Agarwal ; Sudha Agrawal ; Anju Pradhan 等
期刊名称：Nepal Journal of Dermatology, Venereology & Leprology
印刷版ISSN：2091-0231
出版年度：2019
卷号：17
期号：1
语种：English
出版社：Society of Dermatologists, Venereologists and Leprologists of Nepal (SODVELON)
摘要：Progressive symmetrical erythrokeratodermia (PSEK) is a rare autosomal dominant genodermatosis presenting in infancy or early childhood. An 11-year-old male presented with a history of pruritic, erythematous, scaly, hyperkeratotic plaques first noted at 5 years of age, with no history of similar lesions in the family. Cutaneous examination revealed multiple, irregularly shaped, erythematous plaques with fine, shiny white, adherent scaling distributed symmetrically over the face, trunk, inguinal area, bilateral axillae and extensor surfaces of limbs. Palmoplantar keratoderma was present with thickened, yellowish discolored nails. No systemic abnormality was found. The histopathological findings were consistent with PSEK. The case is being reported to increase the awareness about this rare disease.
关键词：Genetic Heterogeneity;Keratoderma;palmoplantar;Nepal;Retinoids