Cell-mediated immunity in Darier's disease: effect of systemic retinoid therapy.

In five patients with Darier's disease, lymphocyte subpopulations, lymphocyte responsiveness to phytohaemagglutinin (PHA), concanavalin A (Con A), pokeweed mitogen (PWM) and purified protein derivative of tuberculin (PPD), leukocyte migration inhibitory factor (LMIF) production and suppressor cell activities were studied before and during oral etretinate treatment. Pre-therapy investigations of cell-mediated immunity showed no severe immunological dysfunction, although high responses to supra-optimal Con A concentrations suggested abnormalities in immunoregulatory lymphocyte subpopulations. In addition, two patients showed enhanced LMIF production upon stimulation with Con A, PWM and PPD. Retinoid therapy decreased the number of peripheral blood total leukocytes, lymphocytes and T-cells, normalized the LMIF production, and decreased the lymphocyte responses to mitogens. Furthermore, the dose-response curve to Con A changed toward normal and the suppressor cell activity regulating Con A responses tended to increase.