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Pachyonychia congenita ��PC�� is a rare autosomal dominant keratin disorder characterized by hypertrophic nail dystrophy, focal palmoplantar keratoderma and oral leukokeratosis. It is clinically subdivided into two major variants, PC-1 and PC-2, with the involvement of nails, skin, oral mucosa, larynx, hair and teeth. It has been shown that PC-1 is caused by the mutations in keratin 6a and 16 genes, whereas those in keratin 6b and 17 genes lead to the development of PC-2. At present, PC is mainly managed by symptomatic treatment; gene therapy has become a hot spot of investigation��and progress has been made in the application of small interference RNA to the treatment of PC.