Congenital bullous poikiloderma (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 poilkoloderma 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.