Gastrointestinale Polypose mit Melanose der Lippen- und Mundschleimhaut (Peutz-Jeghers’sches Syndrom)

The Peutz-Jeghers Syndrome

“…Like polyposis coli, the Peutz-Jeghers syndrome is occasionally associated with other congenital and sometimes hereditary abnormalities. Apart from bladder and nasal polyps mentioned by Peutz and more recently by Hafter (1954), I have encountered examples (amongst my own patients or in the literature) of associated bronchial adenomatosis, a variety of skeletal and cutaneous defects (especially mild finger clubbing), congenital heart disease, extensive small-intestinal diverticulosis, retarded development, and ovarian disease (Dormandy, 1958;Staley and Schwarz, 1957). Also, like polyposis coli, many cases appear sporadically: and in some members of affected families the pigmentation or the polyposis alone may manifest itself.…”