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Li Suo, Li Zhiliang, Feng Suying Department of Dermatology, Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing 210042, China Corresponding author: Feng Suying, Email: fengsy@pumcderm.cams.cn ��Abstract�� Calcium ion ��Ca++�� can maintain the conformation of desmoglein and enhance desmosomal adhesion, and intracellular calcium ion abnormalities play an important role in the pathogenesis of pemphigus. Currently, it is acknowledged that the pathogenesis of pemphigus is associated with a variety of autoantibodies against keratinocyte?associated antigens, which can activate related signaling pathways, and lead to increases in intracellular calcium ion concentration, followed by activation of protein kinase C, phosphorylation and internalization of adhesion molecules, retraction of keratin, collapse of cytoskeleton and desmosome dissociation. Increases in intracellular calcium ion concentration can also initiate mitochondrial pathway?mediated apoptosis or oncosis. During this pathway, caspases can degrade keratin and cause keratin retraction on the one hand, as well as directly induce abnormalities in the structure and function of desmosomal proteins, and impact on original cytoskeleton structures on the other hand, finally leading to acantholysis and apoptosis, which is called ��apoptolysis��.