Clinical Analysis on 13 Korean Cases of Darier's Disease

Jun, Jin Hee; Lee, Jee Bum; Kim, Seong Jin; Won, Young Ho; Lee, Seung Chul

Abstract

BACKGROUND: Darier's disease is an autosomal dominant disoder in keratinizing process. Clinically, patients present with warty to hyperkeratotic plaques with predilection of seborrheic area.
OBJECTIVES
The purpose of this study was to analyze the clinical findings of Darier's disease in Koreans with literature review.
METHODS
Thirteen cases of Darier's disease were reviewed by retrospective study. We analyzed the patients in terms of their clinical features, such as age, sex, family background, location of present or initial lesions, severity, clinical types, and treatment or prognosis.
RESULTS
Darier's disease in Koreans was predominant in male with a sex ratio between male : female of 10 : 3. Three patients had a family history of Darier's disease among siblings and ancestors. Young patients under 30 years old were 79% with average onset of 24 years old. Typical seborrheic type was found in 9 cases, and flexural and linear/nevoid types were 2 cases, respectively. Characteristic nail deformities were observed in 5 cases, all of them showed V-shaped nicking. Focal types responded well to retinoic acid treatment, while seborrheic type was poor to treatment.
CONCLUSION
We reviewed clinical characteristics of Darier's disease in Koreans patients. Interestingly, male-dominance of Darier's disease in Korean suggests the possibility that genetic patterns vary among countries or races in Darier's disease.