ABSTRACT
Extracutaneous findings ● Esophageal strictures in more than 60% of patients ● Cicatricial strictures may occur more rarely in conjunctivae, urethra and perianal region ● Leukokeratoses in oral mucosa with caries ● Peculiar pseudosyndactyly of the first interphalangeal spaces of hands
Complications and course
Blisters are frequent in infancy and progressively decrease with age. In contrast, poikilodermatous changes are progressive and worsen with age, not only in sun-exposed areas.
