Premium
Kindler Syndrome: Report of a Case with Ultrastructural Study and Review of the Literature
Author(s) -
Patrizi Annalisa,
Pauluzzi Paolo,
Neri Iria,
Trevisan Giusto,
Badiali De Giorgi Lucilla,
Pasquinelli Gianandrea
Publication year - 1996
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/j.1525-1470.1996.tb00709.x
Subject(s) - medicine , epidermolysis bullosa , dermatology , context (archaeology) , atrophy , ultrastructure , pathology , paleontology , biology
Kindler syndrome is characterized by a generalized, progressive polkiloderma with cutaneous atrophy, congenital acral skin blistering, and photosensitivity. Since the first description, approximately 70 cases have been reported worldwide, but ultrastructural studies were performed in only five patients. In none of these patients were biopsies done at birth. In our patient ultrastructural studies were performed both of the blister at birth and of the poikilodermatous and atrophic skin at 6 years of age. Some ultrastructural features in the context of a bullous disease of the newborn that resembles epidermolysis bullosa, should alert investigators to the possibility of Kindler syndrome even in absence of the typical clinical signs.