DARIER-WHITE DISEASE - A REVIEW OF THE CLINICAL-FEATURES IN 163 PATIENTS
DARIER-WHITE DISEASE - A REVIEW OF THE CLINICAL-FEATURES IN 163 PATIENTS
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DOI：
10.1016/0190-9622(92)70154-8
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发表时间：
1992-07-01
影响因子：
13.8
通讯作者：
WILKINSON, JD
中科院分区：
文献类型：
--
作者：
BURGE, SM;WILKINSON, JD
背景：Darier病是一种罕见的、以遗传为主的遗传性皮肤病；目前还没有任何关于这种疾病患者的大型临床研究。目标。我们的目的是记录一大群Darier病患者的临床特征。收集了163名患者的资料。结果：发病年龄多在6~20岁之间。该病好发于脂溢区的皮肤；96%有肢端体征；6%有肥厚性屈曲受累；13%有口腔粘膜损害。没有任何减刑。局部治疗有时能缓解症状，但对疾病的进展没有效果。口服维甲酸有效，但长期治疗耐受性差。结论：虽然Darier病是一个长期的负担，但大多数患者仍能过上相对正常的生活。
Background: Darier's disease is a rare, dominantly inherited genodermatosis; there have not been any large clinical studies of patients with this disease. Objective. Our purpose was to document the clinical features in a large group of patients with Darier's disease.Methods. Data were collected from 163 affected persons.Results: The onset usually occurred between the ages of 6 and 20 years. The disease has a predilection for the skin in seborrheic areas; 96% had acral signs; 6% had hypertrophic flexural involvement; and 13% had oral mucosal lesions. There was no remission. Topical therapy sometimes provided relief of symptoms but had no effect on the progress of the disease. Oral retinoids were effective, but long-term therapy was tolerated poorly. Most patients did not have other medical problems.Conclusion: Although Darier's disease is a chronic and unremitting burden, most patients manage to lead a relatively normal life.