Darier's disease and vitamin A. Concentrations of retinoids in serum and epidermis of untreated patients.

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.