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Introduction: Various inherited or acquired disorders are characterized by palmoplantar kera-toderma hyperkeratosis of hands and feet, and when accompanied with deafness indicates mutations in the gene encoding connexin -26 or a particular mutation (A7445G) of the mito-chondrial t-RNA coded for serine (MT-TS1) is created. Case Report: On skin examination of a 7 year old boy, we observed hyperkeratosis of palm and sole, elbows and knees (Keratoderma of palms &soles with extensor surface of limbs involvement), and found out the history and evidence of sensory-neural deafness since birth-day and he had undergone cochlear implant at the age of two. Thus this patient is placed in a rare group of keratoderma associated with sensory-neural deafness. Since there is no his-tory of similar disease in his pedigree, we suspected the boy had a rare type of keratoderma associated with sensory- neural deafness that has mitochondrial inheritance. In reported cases of this disorder in the world there is no involvement of limb's extensor. So we probably report a new subtype of the rare disorder or a rare separate disorder. Conclusion: The patient is a case with diffuse palmoplantar keratoderma with the involvement of extensor surfaces and sensory-neural deafness that have mitochondrial inheritance that is unlikely to be reported yet. The disease should be controlled with maintenance treatments.