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Erythrokeratodermia variabilis.

Y Maekawa1, S Yasaka

  • 1Department of Dermatology, Kumamoto University Medical School, Honjo 1-1-1, Kumamoto 860, Japan.

The Journal of Dermatology
|August 1, 1977
PubMed
Summary
This summary is machine-generated.

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This case report details a 10-year-old boy with erythrokeratodermia variabilis, a rare skin condition. Treatment with polyethylenglycol 400 and corticosteroid ointment showed slight improvement in hyperkeratotic lesions.

Area of Science:

  • Dermatology
  • Genetics

Background:

  • Erythrokeratodermia variabilis (EKV) is a rare, inherited skin disorder characterized by alternating periods of generalized scaling (erythrokeratodermia) and localized, non-scaling red patches (variabilis).
  • The condition typically presents in infancy or early childhood and can significantly impact quality of life due to its chronic and unpredictable nature.

Observation:

  • A case of a 10-year-old Japanese boy with EKV is presented, highlighting generalized hyperkeratotic and erythematous lesions persisting since 3 months of age.
  • Clinical presentation included hyperkeratotic, verrucous lesions on the auricles and trunk, alongside sharply demarcated, non-elevated erythematous lesions.
  • Laboratory findings were unremarkable, suggesting a primary dermatological basis for the observed symptoms.

Findings:

  • Histopathological examination revealed significant hyperkeratosis with a basket weave pattern, moderate acanthosis, and a subtly thickened granular layer.

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  • These findings are consistent with the characteristic histopathological features of erythrokeratodermia variabilis.

    • Implications:

    • This report contributes to the understanding of erythrokeratodermia variabilis in the Japanese population, supplementing existing literature.
    • The case suggests that topical treatments, such as polyethylenglycol 400 and corticosteroid ointments, may offer mild symptomatic relief for hyperkeratotic lesions in EKV.
    • Further research into the genetic and molecular basis of EKV is warranted to explore more targeted and effective therapeutic strategies.