Lamellar ichthyosis: Response to etretinate with transglutaminase 1 recovery

We studied a case of typical lamellar ichthyosis before and after etretinat e treatment for the expression of transglutaminase 1 (TGK) and the presence or absence of the marginal band. Before the treatment, TGK was undetectabl e, although two other components of the marginal band, loricrin and involuc rin, were detected by immunostaining in a normal pattern. The marginal band of the corneocytes was either thin and irregular or completely absent by e lectron microscopic study. After therapy with etretinate, 50 mg/day, the pa tient's skin improved, and biopsies were taken at 4 and 8 months. Transglut aminase 1 became detectable by immunostaining. The marginal band was stilt absent in most corneocytes.