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We describe a case of bullous congenital ichthyosiform erythroderma (epidermolytic hyperkeratosis) with severe transgradient type of palmoplantar keratoderma. It occurred in a thirty year old man, who was born out of first degree consanguineous marriage. Our case had epidermolytic hyperkeratosis probably of subtype PS-1, however, transgradient palmoplantar keratoderma in this patient was a peculiar manifestation.
