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Related Experiment Videos

Cornifying Darier's disease.

R Katta1, J Reed, J E Wolf

  • 1Department of Dermatology, Baylor College of Medicine, Houston, Texas, USA.

International Journal of Dermatology
|December 21, 2000
PubMed
Summary
This summary is machine-generated.

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Darier's disease is a rare genetic skin disorder characterized by keratotic papules and plaques. Acitretin showed mild improvement in a patient with Darier's disease after other treatments failed.

Area of Science:

  • Dermatology
  • Genetics
  • Histopathology

Background:

  • Darier's disease is an autosomal dominant genodermatosis.
  • It is characterized by abnormal keratinization, leading to skin lesions.

Observation:

  • A 48-year-old man presented with a lifelong history of keratotic papules and plaques, primarily affecting the lower legs, with exacerbations in summer.
  • Physical examination revealed extensive hyperkeratotic plaques on the legs, with scattered papules on the trunk, scalp, and extremities.
  • Nail changes included distal notching and red longitudinal streaks.

Findings:

  • Histopathological examination of a leg plaque showed a papillomatous, hyperkeratotic lesion with suprabasal acantholysis and dyskeratosis (corps ronds and grains).
  • These findings are characteristic of Darier's disease.

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  • Previous treatments with topical agents, antimicrobials, and isotretinoin were unsuccessful.

    • Implications:

    • Acitretin was initiated for treatment, showing mild improvement after 3 months.
    • This case highlights the challenges in managing Darier's disease and suggests acitretin as a potential therapeutic option.
    • Further research into effective treatments for Darier's disease is warranted.