[Congenital bullous poikiloderma (Kindler syndrome)].

[kindler syndrome]

We report on an 18 years old female patient who presented with synechia of the labia . History reveals congenital blistering with acral localization and photosensitivity in childhood . At present , the patient showed wide -spread poikoloderma with reticulate hyperpigmentation , xerosis , proximal synechia between fingers and toes and absence of dermatoglyphics , suggestive for congenital bullous poikiloderma (Kindler syndrome ). The diagnosis was confirmed by histology and electron microscopy . Kindler syndrome is a rare , autosomal recessive disorder with synechia of mucosal areas being the presenting symptom .