Systemic retinoids in the management of ichthyoses and related skin types

Abstract

The term retinoid includes both natural and synthetic derivatives of vitamin A. Retinoid-containing treatments have been used since ~1550BC by the early Egyptians. Treatment of ichthyosiform disorders with retinoids dates back at least to the 1930s. Early use of high-dose vitamin A demonstrated efficacy, but because vitamin A is stored in the liver, toxicity limited usefulness. Interest turned to synthetic retinoids in an effort to enhance efficacy and limit toxicity. Acetretin, isotretinoin and, in the past etretinate, have provided the most effective therapy for ichthyosiform conditions. They have been used for a variety of ages, including in newborns with severe ichthyosis and for decades in some patients. Careful surveillance and management of mucous membrane, laboratory, skeletal, and teratogenic side effects has made systemic retinoids the mainstay of therapy for ichthyosis and related skin types.

Figure 1

Figure 1a. A patient with autosomal recessive congenital ichthyosis-lamellar ichthyosis type before treatment with oral retinoid therapy. (We are grateful to Dr. Robert Gruber for providing clinical images.) Figure 1b. After 4 weeks of therapy with acitretin at 30 mg/day. Figure 1c. After 8 weeks of therapy with acitretin at 30 mg/day. Figure 1d. Twelve weeks after discontinuation of acitretin involvement with lamellar scale has returned.

Figure 1

Figure 1a. A patient with autosomal recessive congenital ichthyosis-lamellar ichthyosis type before treatment with oral retinoid therapy. (We are grateful to Dr. Robert Gruber for providing clinical images.) Figure 1b. After 4 weeks of therapy with acitretin at 30 mg/day. Figure 1c. After 8 weeks of therapy with acitretin at 30 mg/day. Figure 1d. Twelve weeks after discontinuation of acitretin involvement with lamellar scale has returned.

Figure 1

Figure 1a. A patient with autosomal recessive congenital ichthyosis-lamellar ichthyosis type before treatment with oral retinoid therapy. (We are grateful to Dr. Robert Gruber for providing clinical images.) Figure 1b. After 4 weeks of therapy with acitretin at 30 mg/day. Figure 1c. After 8 weeks of therapy with acitretin at 30 mg/day. Figure 1d. Twelve weeks after discontinuation of acitretin involvement with lamellar scale has returned.

Figure 1

Figure 1a. A patient with autosomal recessive congenital ichthyosis-lamellar ichthyosis type before treatment with oral retinoid therapy. (We are grateful to Dr. Robert Gruber for providing clinical images.) Figure 1b. After 4 weeks of therapy with acitretin at 30 mg/day. Figure 1c. After 8 weeks of therapy with acitretin at 30 mg/day. Figure 1d. Twelve weeks after discontinuation of acitretin involvement with lamellar scale has returned.

Figure 2

Figure 2a. Epidermolytic ichthyosis (EI) of back before treatment with isotretinoin. Note thick, hystrix type of hyperkeratosis. Figure 2b. EI of back on isotretinoin therapy. The thick hyperkeratosis has been shed and has left a more normal, pliable skin surface.

Figure 2

Figure 2a. Epidermolytic ichthyosis (EI) of back before treatment with isotretinoin. Note thick, hystrix type of hyperkeratosis. Figure 2b. EI of back on isotretinoin therapy. The thick hyperkeratosis has been shed and has left a more normal, pliable skin surface.

Figure 3

Figure 3a. PPK with honeycomb appearance and pseudo-ainhum before acitretin. Figure 3b. During treatment with acitretin, the pseudo-ainhum is improved.

Figure 3

Figure 3a. PPK with honeycomb appearance and pseudo-ainhum before acitretin. Figure 3b. During treatment with acitretin, the pseudo-ainhum is improved.