Rothmund-Thomson Syndrome Medication

Updated: Jun 18, 2018
  • Author: Sylvia Hsu, MD; Chief Editor: Dirk M Elston, MD  more...
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Medication

Medication Summary

The goals of pharmacotherapy for Rothmund-Thomson syndrome (poikiloderma congenitale) are to reduce morbidity and to prevent complications.

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Retinoid-like Agents

Class Summary

These agents decrease the cohesiveness of abnormal hyperproliferative keratinocytes, and they may reduce the potential for malignant degeneration. They also modulate keratinocyte differentiation.

Isotretinoin (Amnesteem, Claravis, Myorisan, Sotrel)

Isotretinoin is a synthetic 13-cis isomer of the naturally occurring tretinoin (trans-retinoic acid). It is approved for use in severe recalcitrant nodular acne.

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.

Tretinoin topical (Atralin, Refissa, Renova, Avita, Retin-A)

Tretinoin inhibits microcomedo formation and eliminates lesions. It makes keratinocytes in sebaceous follicles less adherent and easier to remove. Tretinoin topical is available as 0.025%, 0.05%, and 0.1% creams and 0.01% and 0.025% gels.

Acitretin (Soriatane)

Acitretin is a metabolite of etretinate and related to both retinoic acid and retinol (vitamin A). Its mechanism of action is unknown. However, it is thought to exert therapeutic effect by modulating keratinocyte differentiation, keratinocyte hyperproliferation, and tissue infiltration by inflammatory cells. Acitretin is approved for the treatment of severe psoriasis.

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Keratolytic Agents

Class Summary

This agent causes cornified epithelium to swell, soften, macerate, and then desquamate.

Salicylic acid topical (Clean & Clear Advantage, Neutrogena)

By dissolving the intercellular cement substance, salicylic acid produces desquamation of the horny layer of skin, while not affecting the structure of viable epidermis.

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