Pandapatan Aznaida T, Tan Cindy J, Tan Joyce Anne S
Department of Dermatology, Philippine General Hospital, University of the Philippines Manila.
Acta Med Philipp. 2024 Sep 30;58(17):83-87. doi: 10.47895/amp.v58i17.7957. eCollection 2024.
We report a case of a Filipino child who presented with yellowish hyperkeratotic plaques on the palms and soles with palmar transgredient extension to the wrists, a yellowish hyperkeratotic plaque over the coccygeal area, and brownish-black hyperkeratotic perianal plaques. Patient had delayed physical development and short stature, but no intellectual disability. Histopathologic examination showed palmoplantar keratoderma. These clinical findings of symmetrical palmoplantar keratoderma with periorificial keratotic plaques were consistent with Olmsted Syndrome. Oral retinoids with topical keratolytics afforded significant improvement with increased hand mobility. Although there is no curative management for these patients, current experimental therapies like epidermal growth factor receptor (EGFR) inhibitors and Transient Receptor Potential Vanilloid-3 (TRPV3) antagonists are promising. Olmsted Syndrome is a rare genodermatosis with 73 cases officially reported as of this writing. This is the first case to be reported from the Philippines.
我们报告一例菲律宾儿童病例,该患儿手掌和足底出现淡黄色角化过度斑块,掌部病变向腕部延伸,尾骨区域有一个淡黄色角化过度斑块,肛周有棕黑色角化过度斑块。患儿身体发育迟缓且身材矮小,但无智力障碍。组织病理学检查显示掌跖角化病。这些对称性掌跖角化病伴口周角化过度斑块的临床发现与奥姆斯特德综合征相符。口服维甲酸类药物联合外用角质松解剂使手部活动能力增加,病情有显著改善。虽然这些患者尚无治愈性治疗方法,但目前如表皮生长因子受体(EGFR)抑制剂和瞬时受体电位香草酸亚型3(TRPV3)拮抗剂等实验性疗法很有前景。奥姆斯特德综合征是一种罕见的遗传性皮肤病,截至撰写本文时,官方共报告了73例。这是菲律宾报告的首例病例。