Darier's disease - response to oral Vitamin A: report of a case and brief review

Abstract

Darier's disease is an uncommon chronic dermatosis of autosomal dominant inheritance with significant psychosocial morbidity and shows unsatisfactory response to several topical and systemic therapies or various resurfacing or surgical procedure. A 24-year-old woman having characteristic asymptomatic and progressive, hyperkeratotic papular lesions involving the scalp, face, upper trunk, and dorsal hands and feet was diagnosed clinicopathologically as Darier's disease. She was treated successfully with oral Vitamin A 25000 IU given twice daily. The response was apparent within 4 weeks and most lesions cleared in 2 months without any adverse effects. Oral Vitamin A provides an effective and safe low-cost therapeutic alternative in Darier's disease, especially when systemic retinoids, the only effective treatment, remain contraindicated or is not tolerated and for maintenance therapy in the long-term.

Keywords

• darier-white disease
• keratosis follicularis
• retinoids
• vitamin a