Research in Medicine

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A 13 years old boy with progressive poikiloderma, bullous lesions in the extremities and photosensitivity is reported. Physical examinations were otherwise normal, his physical development was normal, no other family member had a similar disease. Routin laboratory exams were in the normal limits.Histopathology exams from poikilodermatous skin showed atrophy of the epidermis, liquification degeneration of basal lyer and increase in melanin pigment in that area. Biopsy from bullous lesions showed a subepidermal bulla. Poikiloderma is a rare skin sign and when it occurs early in life usually is a sign of a hereditary and congenital disease. Bullous lesions in the extremities which are probably related to the trauma suggest diagnosis of epidermolysis bullousa. Association of the above two signs under the title of Kindler Syndrome was reported by the T. Kindler un 1954 for the fir st time. As far as we 􀁇w this is the first case of Kindler Syndrome which is reported in Iran.

Keywords: Kindler Syndrome

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