Kindler syndrome.

[kindler syndrome]

Kindler syndrome is a rare autosomal recessive disorder associated with skin fragility . It is characterized by blistering in infancy , photosensitivity and progressive poikiloderma . The syndrome involves the skin and mucous membrane with radiological changes . The genetic defect has been identified on the short arm of chromosome 20 . This report describes an 18 -year -old patient with classical features like blistering and photosensitivity in childhood and the subsequent development of poikiloderma . The differential diagnosis of Kindler syndrome includes diseases like Bloom syndrome , Cockayne syndrome , dyskeratosis congenita , epidermolysis bullosa , Rothmund-Thomson syndrome and xeroderma pigmentosum . Our patient had classical cutaneous features of Kindler syndrome with phimosis as a complication .