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Archive

Spring 2005, Vol. 13 No. 1

H.K. Dermatol. Venereol. Bull. (2005) 13, 7-13


Original Article

Darier's disease: survey of 32 Chinese patients

YH Chan 陳耀海 and WYM Tang 鄧旭明

Abstract
A multi-clinic, 20-year, retrospective study had been carried out to review the clinico-pathological features of Chinese patients with Darier's disease (DD) in the Social Hygiene Service. The incidence of DD in Hong Kong was 0.025 per 100,000 per year. The peak age of onset was in the second decade of life with an average of 15. Male to female ratio was around 1:1. Twenty (62%) patients had positive family history. The major symptom experienced was itching. Keratotic papules were invariably present in our patients and mixed pattern with seborrhoeic plus flexural involvement was the commonest phenotypic variation. All of them had acral signs; 38% had oral mucosal lesions. Lesions were most frequently found on face, neck, front chest and ears. There was no full remission. Systemic retinoid was the most effective therapy for our patients although minor side effects were common.