Pettit Cory, Ulman Catherine A, Spohn Gina, Kaffenberger Jessica
Division of Dermatology, The Ohio State University Wexner Medical Center, Columbus, Ohio.
Dermatol Online J. 2018 Mar 15;24(3):13030/qt2827h6qq.
Darier disease is a rare autosomal dominant disorder that results from a mutation in the gene coding for the endoplasmic reticulum membrane calcium pump Ca2+-ATPase type 2 (SERCA2), leading to compromised intercellular adhesion. Patients typically present in the first two decades of life with keratotic, greasy papules in a seborrheic distribution. Segmental Darier disease is a variant with localized disease that follows Blaschko lines. Treatment options include topical and systemic agents including corticosteroids, retinoids, and antibiotics. We present a 67-year-old woman who came to our clinic with segmental Darier disease recalcitrant to topical therapy. Owing to cost and side effect profile, the patient declined treatment with oral retinoids. Doxycycline 100mg daily was started with significant improvement. Tetracyclines both chelate and assist calcium to cross membranes. This mechanism may correct the cellular calcium imbalance present in Darier disease. In addition, tetracyclines have been shown to inhibit metalloproteinase 9, an important part of Darier disease pathogenesis. Owing to its favorable side effect profile, further investigation is warranted to establish doxycycline as a more widely utilized treatment option for Darier disease.
Darier病是一种罕见的常染色体显性疾病，由编码内质网膜钙泵2型（SERCA2）的基因突变引起，导致细胞间黏附受损。患者通常在生命的前二十年出现，在脂溢性分布区域有角化性、油腻性丘疹。节段性Darier病是一种局限性疾病的变体，沿Blaschko线分布。治疗选择包括局部和全身用药，如皮质类固醇、维甲酸和抗生素。我们报告一名67岁女性，因节段性Darier病前来我院就诊，局部治疗无效。鉴于成本和副作用，患者拒绝口服维甲酸治疗。开始每天服用100mg强力霉素，病情有显著改善。四环素既能螯合钙又能协助钙穿过细胞膜。这种机制可能纠正Darier病中存在的细胞钙失衡。此外，四环素已被证明能抑制金属蛋白酶9，这是Darier病发病机制的重要组成部分。由于其良好的副作用，有必要进一步研究以确定强力霉素作为Darier病更广泛使用的治疗选择。
