A Case Report of Keratoderma and Bilateral Deafness

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. 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. (Sci J Hamadan Univ Med Sci 2013; 20 (4): 342-345)