Larangeira de Almeida Hiram, Happle Rudolf, Blume-Peytavi Ulrike, de Castro Luis Antônio Suita
Catholic and Federal University of Pelotas, Pelotas, Brazil.
J Am Acad Dermatol. 2008 Mar;58(3):505-7. doi: 10.1016/j.jaad.2007.03.034.
A 22-year-old woman had an unusual congenital skin disorder in the form of ichthyosis characterized by innumerable keratotic plugs, resulting in a peculiar sievelike appearance. On histopathologic examination the epidermis showed a pronounced orthohyperkeratosis. The keratotic plugs seemed to emerge from the infundibular region of hair follicles. Immunohistochemical characterization revealed a normal keratin and filaggrin expression. Oral treatment with isotretinoin resulted in a reduction of the hyperkeratosis. Because we could not find any previously reported similar case, we propose the new name "ichthyosis cribriformis," which means "sievelike ichthyosis."
一名22岁女性患有鱼鳞病形式的罕见先天性皮肤病，其特征为无数角质栓，导致外观呈奇特的筛状。组织病理学检查显示表皮有明显的正角化过度。角质栓似乎从毛囊漏斗部区域长出。免疫组织化学特征显示角蛋白和丝聚蛋白表达正常。口服异维甲酸治疗使角化过度减轻。由于我们未发现之前报道过的类似病例，我们提出新名称“筛状鱼鳞病”，意思是“筛状的鱼鳞病”。
