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Darier's disease in Singapore
The British Journal of Dermatology
|February 25, 2005
Summary
Darier's disease in Asians presents similarly to Caucasians in incidence and onset. However, Asian patients show unique features like guttate leucoderma and a poor response to topical retinoids.
Area of Science:
- Dermatology
- Genetics
- Rare Diseases
Background:
- Darier's disease is a rare, inherited skin disorder.
- Clinical data on Asian populations are limited compared to Caucasians.
Purpose of the Study:
- To characterize the demographic and clinical features of Darier's disease in Asian patients.
- To compare these findings with existing data from Caucasian populations.
Main Methods:
- Retrospective analysis of 24 non-related Darier's disease cases over 20 years (1982-2002).
- Data collected on patient demographics, clinical presentation, disease triggers, and treatment response.
Main Results:
- Incidence rate of 3.1 per million per decade.
- Predominantly affected males (19/24), with peak onset between 11-20 years.
- Common sites: seborrheic areas (19/24), hands (palmar pits, acrokeratosis, nail changes).
- Sun exposure exacerbated symptoms in 13 patients; 3 had neuropsychiatric disorders.
- Guttate leucoderma observed in 3 patients; haemorrhagic macules were absent.
- Oral retinoids effective; topical retinoids showed poor response.
Conclusions:
- Asian Darier's disease shares similarities with Western cohorts regarding incidence, age of onset, and neuropsychiatric associations.
- Distinctive features in Asians include guttate leucoderma, rarity of acrokeratosis, absence of haemorrhagic macules, and limited efficacy of topical retinoids.