Clinical manifestation

Keratotic lesions confined to palms and soles; thick, horny, hard, yellowish plaques with waxy smooth surfaces; plaques sometimes pitted and verrucous, surrounded by erythematous halos; occasional corneal opacites; pili torti, sensorineural hearing loss; hypohidrosis; dental abnormalities

Differential diagnosis Mal de Meleda; Papillon-Lefevre syndrome; hereditary epidermolytic palmoplantar ker-atoderma; Vohwinkel syndrome; Richner-Hanhart syndrome; progressive kerato-derma; punctate keratoderma; pityriasis rubra pilaris; xerosis

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