Erythrokeratodermia Variabilis et Progressiva  Medication

Updated: May 30, 2017
  • Author: Gabriele Richard, MD, FACMG; Chief Editor: Dirk M Elston, MD  more...
  • Print
Medication

Medication Summary

The goals of pharmacotherapy in erythrokeratodermia variabilis et progressiva (EKVP) are to reduce morbidity and prevent complications.

Next:

Retinoid-like Agents

Class Summary

Systemic retinoid therapy can induce dramatic improvement. Long-term therapy is required to achieve continuing results. The minimal effective dose for persons with EKVP is usually very low.

Acitretin (Soriatane)

Acitretin is a retinoic acid analog similar to etretinate and isotretinoin. Etretinate is the main metabolite; it has demonstrated clinical effects similar to those of etretinate. The mechanism of action is unknown. For EKVP, the effect of acitretin or etretinate is superior to that of isotretinoin. The minimal effective dose for persons with EKVP usually is very low. Treatment may be started at a very low dose (10 mg/day or less) and adjusted based on the therapeutic effect, which begins 1-2 weeks after initiating systemic retinoid therapy. The use of retinoids should be considered carefully because long-term therapy is required to achieve continuing results.

Isotretinoin (Amnesteem, Claravis, Sotret)

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. For EKVP, the effect of acitretin or etretinate is superior to that of isotretinoin.

The minimal effective dose for persons with EKVP usually is very low. Treatment may be started at low dose (0.5 mg/kg/day) and adjusted based on therapeutic effect, which begins 1-2 weeks after initiating systemic retinoid therapy. The use of retinoids should be considered carefully because long-term therapy is required to achieve continuing results.

Previous
Next:

Antihistamines, 1st Generation

Class Summary

These agents are used to prevent the histamine response in sensory nerve endings and blood vessels. They are more effective in preventing histamine response than in reversing it.

Diphenhydramine (Benadryl, Aler-Dryl, Diphen, AllerMax)

Diphenhydramine is used for symptomatic relief of pruritus and burning caused by the release of histamine.

Cyproheptadine

Cyproheptadine is used for the symptomatic relief of allergic symptoms caused by histamine released in response to allergens and skin manifestations.

Hydroxyzine (Vistaril)

Hydroxyzine antagonizes H1 receptors in the periphery. It may suppress histamine activity in the subcortical region of the CNS.

Previous