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Erythrokeratoderma variabilis: case report and review of the literature
R C Knipe1, F P Flowers, F R Johnson
1Department of Dermatology, University of Texas Medical Branch, Galveston 77555, USA.
Pediatric Dermatology
|March 1, 1995
Insights
This case study presents a 5-month-old boy with erythrokeratoderma variabilis, a rare skin condition. Topical retinoic acid treatment was ineffective for the fluctuating erythematous plaques.
Area of Science:
- Dermatology
- Pediatric Dermatology
- Genodermatoses
Background:
- Erythrokeratoderma variabilis (EKV) is a rare autosomal dominant genodermatosis.
- It is characterized by transient erythematous (red) and hyperkeratotic (thickened skin) plaques.
- EKV presents with significant clinical variability, even within families.
Purpose of the Study:
- To present a case of erythrokeratoderma variabilis in a 5-month-old infant.
- To highlight the clinical presentation and course of the condition.
- To discuss the limited efficacy of topical retinoic acid in this case.
Main Methods:
- Clinical case presentation.
- Observation of disease progression over hours to days.
- Review of existing literature on erythrokeratoderma variabilis.
Main Results:
- A 5-month-old boy exhibited asymptomatic, erythematous plaques.
- The skin lesions demonstrated rapid changes in morphology and distribution.
- Topical retinoic acid therapy did not resolve the plaques.
Conclusions:
- Erythrokeratoderma variabilis can manifest in early infancy.
- The fluctuating nature of lesions is a key diagnostic feature.
- Treatment options for EKV may require further investigation beyond topical retinoic acid.
Abstract:
A 5-month-old boy with erythrokeratoderma variabilis is presented. The parents noted that the asymptomatic erythematous plaques changed over the course of hours to days. Topical therapy with retinoic acid proved ineffective. A brief review of the literature is presented.