Two cases with Papillon-Lefèvre Syndrome (PLS) are reported. They are two brothers belonging to a family in which there is strong consanguinity. An autosomal recessive pattern of inheritance is suggested. Some pathological findings as congenital bronchiectasis observed in one of the patients as well as the PLS features are discussed and considered to be fortuitous defects. These observations are compared with those described in the literature.
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