As a rare and refractory hereditary skin disease belonging to the familial and autosomal dominant dermatosis, the Vohwinkel syndrome (VS) is characterized by diffuse hyperkeratosis in the palms of hands and soles of feet, which would develop to auto-amputation if not treated adequately. 1,2 This disease is usually accompanied by many other medical disorders, for example sensorineural deafness, ichthyosis, vitiligo and mental retardation, severely affecting the patients' physical and mental health. 2 However, the effective therapeutic treatment of VS is still lacking in the clinical settings. 3 Recent studies have shown that there are two types of palmoplantar keratoderma mutilating. One is an ichthyosis-related variant caused by the insertional mutations in the loricrin gene. [4][5][6] The other