Vahlquist A, Lee J B, Michaëlsson G
Arch Dermatol. 1982 Jun;118(6):389-92. doi: 10.1001/archderm.118.6.389.
Blood samples and skin biopsy specimens from 13 male patients with Darier s disease (DD) and 11 healthy male controls were analyzed for carotene, retinol (vitamin A), dehydroretinol (vitamin A2), and retinol-binding protein (RBP). The serum carotene level was lower in the patients than in the controls, but the vitamin A and RBP levels did not differ from those of the controls regardless of the severity of the disease. The RBP could be visualized by immunofluorescence microscopy in the epidermis of both unaffected and lesional skin of patients with DD. In the epidermis of patients with DD (uninvolved and lesional), the carotene content was similar to that of the controls. The retinol content was probably higher than in the controls. By contrast, the dehydroretinol concentration was normal in the uninvolved skin but markedly higher than normal in the lesions, especially in the severe cases. The abnormal distribution of retinoids in DD may reflect localized disturbances of epidermal vitamin A metabolism rather than a defect in serum vitamin A transport.
对13例 Darier病（DD）男性患者和11名健康男性对照者的血样及皮肤活检标本进行了胡萝卜素、视黄醇（维生素A）、脱氢视黄醇（维生素A2）和视黄醇结合蛋白（RBP）分析。患者血清胡萝卜素水平低于对照组，但无论疾病严重程度如何，维生素A和RBP水平与对照组无差异。通过免疫荧光显微镜可在DD患者未受累及病变皮肤的表皮中观察到RBP。在DD患者（未受累及病变）的表皮中，胡萝卜素含量与对照组相似。视黄醇含量可能高于对照组。相比之下，脱氢视黄醇浓度在未受累皮肤中正常，但在病变中明显高于正常，尤其是在严重病例中。DD中类视黄醇的异常分布可能反映了表皮维生素A代谢的局部紊乱，而非血清维生素A转运缺陷。
