Wang Jason F., Lederhandler Margo H., Brinster Nooshin, Soter Nicholas A.
J Drugs Dermatol. 2019 Feb 1;18(2):213-214.
Darier disease is an autosomal dominant genodermatosis of abnormal keratinization characterized by hyperkeratotic papules and plaques with a predilection for seborrheic areas. We report a case of a rare vesiculobullous variant of treatment-resistant Darier disease in a 55-year-old woman that failed topical tacrolimus and topical and oral glucocorticoids. Cetirizine was initiated at 10 mg daily and increased to 40 mg daily over four weeks, with resultant marked improvement of the patient’s burning sensation. A punch biopsy revealed a perivascular infiltrate of eosinophils. This patient’s symptomatic improvement with cetirizine, which has antagonizing properties against eosinophils, highlights the potential role of eosinophils in the pathogenesis of vesiculobullous Darier disease. We suggest that major basic protein secreted by eosinophils may propagate blister formation in vesiculobullous Darier disease by disrupting desmosomes. J Drugs Dermatol. 2019;18(2):213-214.
Darier病是一种常染色体显性遗传性角化异常性皮肤病，其特征为角化过度性丘疹和斑块，好发于脂溢部位。我们报告了一例55岁女性的罕见水疱大疱型难治性Darier病，该患者外用他克莫司以及外用和口服糖皮质激素治疗均无效。开始给予西替利嗪每日10mg，四周内增至每日40mg，患者的烧灼感明显改善。皮肤活检显示血管周围有嗜酸性粒细胞浸润。该患者使用具有嗜酸性粒细胞拮抗特性的西替利嗪后症状改善，突出了嗜酸性粒细胞在水疱大疱型Darier病发病机制中的潜在作用。我们认为嗜酸性粒细胞分泌的主要碱性蛋白可能通过破坏桥粒在水疱大疱型Darier病中促使水疱形成。《皮肤药物学杂志》。2019年；18(2):213 - 214。
