Amagai Masayuki
Department of Dermatology, Keio University School of Medicine, Tokyo, Japan.
Vet Dermatol. 2009 Oct;20(5-6):308-12. doi: 10.1111/j.1365-3164.2009.00831.x.
Desmosomes are intercellular adhesive junctions of epithelial cells that contain two major transmembrane components, desmogleins (Dsg) and desmocollins; these are both cadherin-type cell-cell adhesion molecules. Pemphigus is an autoimmune blistering disease caused by IgG autoantibodies that target Dsg1 and Dsg3 in pemphigus foliaceus and pemphigus vulgaris respectively. Bullous impetigo is a common and highly contagious superficial skin infection caused by Staphylococcus aureus. Staphylococcal scalded skin syndrome (SSSS) is a generalized form of bullous impetigo. The blisters in bullous impetigo and SSSS are induced by exfoliative toxin that specifically cleaves Dsg1. Clinical and microscopic localization of blisters in pemphigus, bullous impetigo and SSSS are logically explained at the molecular level by the desmoglein compensation theory; the similarity of lesions among these diseases is underscored by a similar pathogenesis.
桥粒是上皮细胞的细胞间黏附连接,包含两种主要的跨膜成分,桥粒芯糖蛋白(Dsg)和桥粒胶蛋白;它们都是钙黏蛋白型细胞间黏附分子。天疱疮是一种自身免疫性水疱病,由IgG自身抗体引起,在落叶型天疱疮和寻常型天疱疮中,这些抗体分别靶向Dsg1和Dsg3。大疱性脓疱疮是由金黄色葡萄球菌引起的常见且具有高度传染性的浅表皮肤感染。葡萄球菌性烫伤样皮肤综合征(SSSS)是大疱性脓疱疮的一种全身性形式。大疱性脓疱疮和SSSS中的水疱是由特异性切割Dsg1的剥脱毒素诱导产生的。桥粒芯糖蛋白补偿理论在分子水平上从逻辑上解释了天疱疮、大疱性脓疱疮和SSSS中水疱的临床和微观定位;这些疾病之间病变的相似性通过相似的发病机制得到了强调。