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Erythrokeratodermia Variabilis et Progressiva  Follow-up

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

Further Outpatient Care

Erythrokeratodermia variabilis et progressiva (EKVP) patients receiving systemic retinoid therapy should be followed up on a regular basis to monitor treatment effectiveness and adverse effects. Establishing a long-term relationship with patients is important in re-evaluating effectiveness of topical therapy and in directing the families to appropriate resources for social and family support.

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Complications

Complications are rare with erythrokeratodermia variabilis et progressiva (EKVP). Complications mainly involve the adverse effects of systemic retinoid therapy. Heat intolerance may occur in individuals with generalized hyperkeratosis.

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Prognosis

Erythrokeratodermia variabilis et progressiva (EKVP) is a chronic skin disorder without other organ manifestations; patients have a normal life expectancy. If properly treated, the skin manifestations of EKVP can be well controlled. Depending on the extent and severity of erythrokeratodermia variabilis, the skin lesions also can be severely disfiguring and have a tremendous psychosocial effect on the patients. In addition, the erythematous patches may diminish as the patient ages. Generalized hyperkeratosis may be associated with heat intolerance.

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Patient Education

Patients of childbearing age should receive genetic counseling. Molecular diagnostic testing for mutations in the connexin genes GJB3,GJB4, and GJA1 is available. The results of such analysis will allow for appropriate genetic counseling of the patient and family, particularly with respect to recurrence risk in future pregnancies.

Triggering events, such as exposure to cold, drastic temperature changes, and mechanical irritation of the skin, should be avoided. Patients with generalized hyperkeratosis should be informed about the possibility of heat intolerance.

The Foundation for Ichthyosis and Related Skin Types, F.I.R.S.T., is a patient advocacy group that offers information, educational materials, and support for patients and their families.

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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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