Thomas D R, Jorizzo J L, Brysk M M, Tschen J A, Miller J, Tschen E H
Arch Dermatol. 1984 Nov;120(11):1475-9. doi: 10.1001/archderm.120.11.1475.
Two patients, a father and son, with pachyonychia congenita were treated with orally administered isotretinoin because the extreme deformity and discomfort associated with their massive keratoderma interfered with their work and school, respectively. While clinical benefits could not be sustained, electron microscopic findings compatible with suppression of abnormal keratinization were observed. In addition, skin biopsy samples were analyzed by sodium dodecyl sulfate-polyacrylamide gel electrophoresis, and the gels were then subjected to a lectin overlay technique with concanavalin A labeled with iodine 125. The distribution of specific glycoproteins was found to be different for lesional as against normal epidermis. The procedure was repeated after oral treatment with isotretinoin. The labeled glycoprotein pattern of the lesional epidermis was clearly distinguishable from both the pretreatment lesional and the normal epidermis; it was mostly intermediate between the two. The normal epidermis was virtually unaffected by the retinoid treatment.
两名患有先天性厚甲症的患者（父子）接受了口服异维甲酸治疗，因为他们大面积的皮肤角化症所带来的极度畸形和不适，分别影响了他们的工作和学业。虽然临床疗效无法持续，但观察到了与异常角化抑制相符的电子显微镜检查结果。此外，通过十二烷基硫酸钠-聚丙烯酰胺凝胶电泳对皮肤活检样本进行分析，然后将凝胶用125碘标记的伴刀豆球蛋白A进行凝集素覆盖技术处理。发现病变表皮与正常表皮相比，特定糖蛋白的分布有所不同。口服异维甲酸治疗后重复该过程。病变表皮的标记糖蛋白模式与治疗前病变表皮和正常表皮均有明显区别；它大多介于两者之间。正常表皮实际上未受维甲酸治疗的影响。
