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Erythrokeratodermia Variabilis et Progressiva  Medication

  • Author: Gabriele Richard, MD, FACMG; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Nov 04, 2015
 

Medication Summary

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

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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.

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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.

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Contributor Information and Disclosures
Author

Gabriele Richard, MD, FACMG Chief Medical Officer, GeneDx, Inc

Gabriele Richard, MD, FACMG is a member of the following medical societies: American Society of Human Genetics, Society for Pediatric Dermatology

Disclosure: Receive salary for employment from GeneDx, a wholly owned subsidiary of BioReference Labs, a wholly owned subsidiary of Opko Health.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Harry Dao, Jr, MD Assistant Professor, Department of Dermatology, Baylor College of Medicine

Harry Dao, Jr, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Mark W Cobb, MD Consulting Staff, WNC Dermatological Associates

Mark W Cobb, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society of Dermatopathology

Disclosure: Nothing to disclose.

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Figurate erythema. Courtesy of M. King and J. Crawford.
Targetlike erythema. Courtesy of M. King and J. Crawford.
Generalized hyperkeratosis with scaling, accentuated skin lines, and figurate erythema. Courtesy of M. King and J. Crawford.
Thick hyperkeratotic plates with hystrixlike spines. Courtesy of M. King and J. Crawford.
Sharply demarcated, figurate, hyperkeratotic plaques in a symmetric distribution. Courtesy of M. King and J. Crawford.
Figurate hyperkeratotic plaque with erythematous patches. Courtesy of M. King and J. Crawford.
Plantar keratoderma with peeling. Courtesy of M. King and J. Crawford.
Diffuse glovelike palmar keratoderma. Courtesy of M. King and J. Crawford.
 
 
 
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