Lamellar Ichthyosis Medication

Updated: Apr 01, 2022
  • Author: Chris G Adigun, MD, FAAD; Chief Editor: Dirk M Elston, MD  more...
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Medication Summary

This disorder has no cure; therefore, treatment is directed at decreasing symptoms. This condition, along with the other congenital ichthyoses, is 1 of the targets in gene therapy research.

Emollients should be applied after showering or bathing. The stratum corneum can absorb 6 times its weight in water, and a heavy emollient, such as petrolatum jelly (Vaseline) or water-in-oil preparations (eg, Eucerin) should be applied while the skin is still wet. Alpha-hydroxy acids, such as lactic acid (eg, Lac-Hydrin), help reduce corneocyte adhesion and decrease the thickness of the epidermis. Urea creams can help soften scales. Salicylic acids in combination with propylene glycol help to remove dark scaling. Care must be taken when using topical salicylates over large areas, especially in children, because of reports of systemic salicylate intoxication. Topical retinoic acids (eg, Retin-A) decrease thickened scaling. Antiseptics and antimicrobials can be used topically to control odor. Because of the significant long-term adverse effects, reserve systemic retinoids for severe disease that is refractory to conventional therapy. Topical botanicals have been used, but more study is needed. [28]

Other therapies that have resulted in clinical improvement are Locobase fatty cream, which is 5% lactic acid and 20% propylene glycol in a lipophilic cream base [29] ; topical N -acetylcysteine, which has an antiproliferative effect [30, 31, 32] ; tazarotene topical 0.05%, a receptor-selective retinoid [33] ; and calcipotriol, a synthetic derivative of vitamin D-3. [34]

25-hydroxyvitamin D3 deficiency is often reported in lamellar ichthyosis patients. Therefore, screening vitamin D levels and supplementing as necessary are recommended. [35]  

Oral liarozole (75-150 mg/day) was reported to help scaling and other symptoms but did not reach statistical significance because of the small sample size. [36]

Alternatively, ex vivo gene therapy has been reported for lamellar ichthyosis, with which normal gene expression of TGM1 has been restored and the phenotype correction was observed in engrafted lesional skin in vivo on the back of immunodeficient mice. Gene therapy serves to be a novel therapeutic approach to lamellar ichthyosis. [10]

Special medication considerations

Many of the medications used for lamellar ichthyosis have a long list of potential adverse effects, and care must be taken to discuss the advantages and disadvantages of treatment. Salicylate toxicity has been reported with systemic absorption of topical salicylic acids in the treatment of children with ichthyosis. [37] A case of lactic acidosis has been reported, with clinical signs of irritability, agitation, myoclonia, and difficulty walking, all which resolved upon discontinuation of the topical treatment. [38]

Topical tacrolimus, a macrolide immunosuppressant, should be used with caution because significantly elevated systemic tacrolimus levels have been reported in a patient with lamellar ichthyosis. [39]

The Pediatric Dermatology Research Alliance (PeDRA) Use of Retinoids in Ichthyosis Work Group has issued recommendations concerning retinoid treatment in lamellar ichthyosis. The Work Group concluded that lamellar ichthyosis has shown improvement with topical or oral steroids, and "both adults and children with moderate to severe disorders of keratinization with significant functional or psychological impairment should be offered the opportunity to make a benefit/risk assessment of treatment with a systemic." [27]


Alpha-hydroxy acids

Class Summary

These agents decrease the thickness of the epidermis and reduce corneocyte adhesion.

Ammonium lactate (Lac-Hydrin)

Ammonium lactate relieves itching and aids healing of skin in mild eczemas and dermatoses, itching skin, minor wounds, and minor skin irritations. The formulation is 12% ammonium lactate in a base containing propylene glycol.


Topical retinoids

Class Summary

These agents appear to decrease the cohesiveness of follicular epithelial cells and stimulate mitotic activity, resulting in an increase in turnover of follicular epithelial cells.

Tretinoin topical (Retin-A, Avita)

Topical tretinoin inhibits microcomedo formation and eliminates lesions. It makes keratinocytes in sebaceous follicles less adherent and easier to remove. Use 0.01% gel.

Tazarotene (Tazorac)

Tazarotene is a topical gel 0.05%. It is a retinoid prodrug whose active metabolite modulates differentiation and proliferation of epithelial tissue; it may also have anti-inflammatory and immunomodulatory properties. Make sure skin is dry before applying gel.


Systemic retinoids

Class Summary

These agents inhibit sebaceous gland function and keratinization.

Isotretinoin (Accutane)

Isotretinoin is an oral agent that treats serious dermatologic conditions. It is a synthetic 13-cis isomer of naturally occurring tretinoin (trans -retinoic acid). Both agents are structurally related to vitamin A. Isotretinoin decreases sebaceous gland size and sebum production. It may inhibit sebaceous gland differentiation and abnormal keratinization.

A US Food and Drug Administration–mandated registry is now in place for all individuals prescribing, dispensing, or taking isotretinoin. For more information on this registry, see iPLEDGE. This registry aims to further decrease the risk of pregnancy and other unwanted and potentially dangerous adverse effects during a course of isotretinoin therapy.

Acitretin (Soriatane)

Acitretin is a retinoic acid analog, similar to etretinate and isotretinoin. Etretinate is the main metabolite and has demonstrated clinical effects similar to those seen with etretinate. The mechanism of action is unknown.