Epidermolytic hyperkeratosis is a form of ichthyosisnor mallyresistant to topical treatments. Female patient monitored since 1978 diagnosed with epidermolytic hyperkeratosis. Clinical examination showed generalized hyperkeratosis and scaling. Given that no other treatments were available at the time, the patient was initially treated with keratolytic, systemic vitamin A and moisturizers, with no improvement. In 1986, with the development of oralretinoids, etretinate was introduced. In 1998 this was replaced by acitretin. The patient is receiving 25 mg/day after 23 years of using oral retinoids. Significant improvement of the condition and patient́s quality of life has been noted. © 2011 by Anais Brasileiros de Dermatologia.864 SUPPL. 17275Zhang, X., He, Y., Zhou, H., Luo, Q., Li, C., Severe ichthyosis-related disorders in children: Response to acitretin (2007) J Dermatolog Treat, 18, pp. 118-122Sousa, P.F., Salas, I.R., Crisóstomo, M.C.C., Oliveira, E.F., Azulay, R.D., Kac, B.K., Hiperceratose epidermolítica em mosaico (2003) An Bras Dermatol, 78, pp. 477-481Verfaille, C.J., Vanhoutte, F.P., Blanchet-Bardon, C., van Steensel, M.A., Steijlen, P.M., Oral liarozole vs. acitretin in the treatment of ichthyosis:A phase II/III multicentre, double-blind, randomized,active-controlled study (2007) Br J Dermatol, 156, pp. 965-973Tey, H.L., Theng, T.S., Acitretin induced bipedal edema (2006) J Dermatol, 33, pp. 372-374Virtanen, M., Gedde-Dahl Jr., T., Mörk, N.J., Leigh, I., Bowden, P.E., Vahlquist, A., Phenotypic/genotypic correlations in patients with epidermolytic hyperkeratosis and the effects of retinoid therapy on keratin expression (2001) Acta Derm Venereol, 81, pp. 161-170Van Dooren-Greebe, R.J., Lemmens, J.A., de Boo, T., Hangx, N.M., Kuijpers, A.L., van de Kerkhof, P.C., Prolonged treatment with oral retinoids in adults: No influence on the fre quency and severity of spinal abnormalities (1996) Br J Dermatol, 134, pp. 71-76McMullen, E.A., McCarron, P., Irvine, A.D., Dolan, O.M., Allen, G.E., Association between long-term acitretin therapy and osteoporosis:No evidence of increased risk (2003) Clin Exp Dermatol, 28, pp. 307-309Zhang, X.B., Luo, Q., Li, C.X., He, Y.Q., Xu, X., Clinical investigation of acitretin in children with severe inherited keratinization disorders in China (2008) J Dermatolog Treat, 19, pp. 221-228Mork, N.J., Austad, J., Kolbenstvedt, A., Bamboo spine mimicking Bekhterev's disease caused by long-term acitretin treatment (2006) Acta Derm Venereol, 86, pp. 452-453Bondeson, M.L., Nyström, A.M., Gunnarsson, U., Vahlquist, A., Connexin 26 (GJB2) mutations in two Swedish patients with atypical Vohwinkel (mutilating keratoderma plus deafness) and KID syndrome both extensively treated with acitretin (2006) Acta Derm Venereol, 86, pp. 503-508Khandpur, S., Bhat, R., Ramam, M., Ichthyosis follicularis, alopecia and photophobia (IFAP) syndrome treated with acitretin (2005) J Eur Acad Dermatol Venereol, 19, pp. 759-762Singh, S., Bhura, M., Maheshwari, A., Kumar, A., Singh, C.P., Pandey, S.S., Successful treatment of harlequin ichthyosis with acitretin (2001) Int J Dermatol, 40, pp. 472-473