PDF(Pubmed)
Abstract：
Darier disease is an autosomal dominant disorder with hyperkeratotic papules affecting primarily seborrheic areas of the upper chest, back, forehead, scalp, nasolabial folds, ears, and, less frequently, the oral mucosa. A typical eruption consists of keratotic and crusted skin-colored papules and plaques. Pruritus occurs in 80% of patients, and pain is rare. Lesions can be triggered by exposure to ultraviolet light, heat, or stress. Secondary infections of the lesions are a common complication. A definitive diagnosis is obtained by a biopsy showing histological features such as acantholysis, suprabasal clefts, and \"corps rond and grains\". Here we present a 37-year-old woman admitted to the gynecology department with pruritic lesions she had noticed on her vulva and perineum for three months. A vulvar biopsy led to the diagnosis of Darier disease. She was referred to the dermatology department and treated with oral acitretin since systemic retinoids are offered as the first-line treatment of the disease.
摘要：
Darier病是一种常染色体显性遗传病，主要影响上胸部的脂溢性区域，回来,前额,头皮,鼻唇沟,耳朵,and,不那么频繁,口腔粘膜。典型的喷发包括角化和结痂的皮肤颜色丘疹和斑块。瘙痒发生在80％的患者中，疼痛是罕见的。暴露于紫外线可以引发病变，热,或压力。病变的继发感染是常见的并发症。明确的诊断是通过活检显示组织学特征，如棘皮松解，基底上裂痕,和“军团红肿和谷物”。在这里，我们介绍了一名37岁的妇女，该妇女入院于妇科，她在外阴和会阴上发现了三个月的瘙痒性病变。外阴活检导致Darier病的诊断。由于提供全身性类维生素A作为该疾病的一线治疗，因此她被转诊到皮肤科，并接受了口服阿曲汀治疗。
