Darier’s disease: hopes and challenges

In an article in the JRSM 14 years ago, SB described the predicament of a patient with Darier’s disease (keratosis follicularis): ‘His scalp is crusted, the skin around his ears weeps and scales... the odour which shadows him pervades the immediate environment and remains when he has gone’. She concluded, ‘Perhaps by the end of this century it will be possible to describe precisely what has happened in the skin in Darier’s disease and this will result in improved therapy so that our patients can lead a normal life.’1 We now discuss the progress made in the intervening years, as it bears on the case of another patient, AB. AB’s disease is extensive, covering the trunk, face, neck and scalp; and severe, being refractory to conventional medical therapy. Erosive flexural disease in the natal cleft causes painful fissuring and secondar