A Very Rare Case of Kindler Syndrome

摘要

Kindler syndrome is a very rare hereditary disorder characterized by acral blister formation in infancy and childhood, progressive poikiloderma, cutaneous atrophy and increased photosensitivity. Since it was first described by Kindler in 1954; less than 100 cases have been reported worldwide. Recently it has been reported that is the first genodermatosis caused by a defect in the actin-extracellular matrix linkage, and the gene was mapped to chromosome 20p12.3. The clinical features of the syndrome have been annotated by different authors but the definite criteria to confirm the diagnosis have not yet been generally accepted.. We report a case that presented to our dermatology department and later on diagnosed as a case of Kindler syndrome at our histopathology department based on clinical as well as on histopathological findings.