Treatment of Darier´s disease with an oral aromatic retinoid (Ro 10-9359): a clinical and light and electron microscopic study

An aromatic retinoid (Ro 10-9359) cleared 6 of 8 patients with Darier's disease in 4-6 weeks. After discontinuing the treatment, remission was maintained for up to 5 months. In 2 severe cases continuous treatment proved necessary in order to maintain the improved state. Acantholysis, various types of dyskeratosis and acanthosis with downward proliferation of rete ridges were seen by light microscopy before treatment. Electron microscopy revealed a decreased number of desmosomes and other cellular attachments and conspicuous microvillous changes of cell surface in separated basal cells. Keratinization in epidermal cells was premature and characterized by abnormal aggregations of keratinization products. Under the influence of retinoid there seemed to appear a new generation of basal cells better able to form intercellular attachments, after which the progress of keratinization in the entire epidermis shifted towards normal, and acanthosis decreased.