漸進對稱性斑角皮症是一個罕見的長皮增生角化疾病,一般它屬於顯性遺傳並有不同臨床表現,但有40%的病人為偶發突變的。通常的服A酸對漸進對稱性紅斑角皮症的治療效槼良好。我們報告一個漸進對稱性紅斑角皮症病例,但此病人對口服A酸,外用類固醇、尿素、A酸及維生素D衍生物等治療,效果都不彰。
Progressive symmetric erythrokeratodermia (PSEK) is a rare disorder disorder of cornification character- ized by epidermal hyperproliferation and is inherited as an autosomal dominant trait with variable pen- etrance. However, it has been reported that sporadic mutations comprise of 40 percent of all cases. Patients with PSEK usually respond to oral retinoid or etretinate therapy. We report a case of progress- sive symmetric erythrokeratodermia that was recalcitrant to oral acitretin and topical steroid, urea and tretinolin, as well as calcipotriol.