KINDLER SYNDROME - REPORT OF A CASE WITH ULTRASTRUCTURAL-STUDY AND REVIEW OF THE LITERATURE

Kindler syndrome is characterized by a generalized, progressive poikil oderma with cutaneous atrophy, congenital acral skin blistering, and p hotosensitivity. Since the first description, approximately 70 cases h ave been reported worldwide, but ultrastructural studies were performe d in only five patients. In none of these patients were biopsies done at birth, In our patient ultrastructural studies were performed both o f the blister at birth and of the poikilodermatous and atrophic skin a t 6 years of age. Some ultrastructural features in the context of a bu llous disease of the newborn that resembles epidermolysis bullosa, sho uld alert investigators to the possibility of Kindler syndrome even in absence of the typical clinical signs.