Related Experiment Videos
Cornifying Darier's disease.
1Department of Dermatology, Baylor College of Medicine, Houston, Texas, USA.
International Journal of Dermatology
|December 21, 2000
Summary
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.
- 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.