Zeglaoui F, Zaraa I, Fazaa B, Houimli S, El Fekih N, Ezzine N, Kamoun M R
Department of Dermatology, Charles Nicolle Hospital, Bd 9 Avril 1006 Tunis, Tunisia.
J Eur Acad Dermatol Venereol. 2005 Jan;19(1):114-7. doi: 10.1111/j.1468-3083.2004.01096.x.
Dyskeratosis follicularis (Darier's disease) is rare autosomal dominant disease characterized by the loss of adhesion between epidermal cells and by abnormal keratinization.
We performed a retrospective study of all the patients diagnosed with Darier's disease at the Department of Dermatology of Charles Nicolle Hospital of Tunis, between 1971 and 2002.
During the observation period, we identified 12 patients with Darier's disease; five males and seven females with a mean age of 17.36 years. No family history was found in eight patients. Skin lesions in the form of keratotic papules were noted in seborrhoeic areas, essentially the face (nine patients), chest and scalp. Seven patients had nail lesions. UV light exposure exacerbated the disease symptoms in seven cases. The patients were treated with topical and systemic retinoids (six cases).
Although Darier's disease has a chronic course, most patients manage to lead a relatively normal life. Treatment is usually unsatisfactory despite much progress in understanding of the underlying abnormalities in Darier's disease.
毛囊角化病（达里埃病）是一种罕见的常染色体显性疾病，其特征为表皮细胞间黏附丧失和异常角化。
我们对1971年至2002年期间在突尼斯查尔斯·尼科勒医院皮肤科诊断为达里埃病的所有患者进行了一项回顾性研究。
在观察期内，我们确定了12例达里埃病患者；5名男性和7名女性，平均年龄为17.36岁。8例患者无家族病史。在脂溢性区域发现角化性丘疹形式的皮肤病变，主要在面部（9例患者）、胸部和头皮。7例患者有指甲病变。7例患者紫外线暴露使疾病症状加重。患者接受了局部和全身维甲酸治疗（6例）。
尽管达里埃病病程慢性，但大多数患者仍能过上相对正常的生活。尽管在理解达里埃病潜在异常方面取得了很大进展，但治疗通常并不令人满意。
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