Congenital erythropoietic porphyria or Günther's disease along with a rare mandibular adenomatoid odontogenic tumor

• Main Authors: Kaushal Mahendra Shah (Author), Amol Karagir (Author), Shridevi Adaki (Author)
• Format: Book
• Published: Wolters Kluwer Medknow Publications, 2016-01-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.

Congenital erythropoietic porphyria (CEP), or "Günther disease," is a rare variant of porphyria. It is an autosomal recessive disease caused by deficient uroporphyrinogen III synthase (URO-III-synthase), the fourth enzyme in the heme biosynthetic pathway. We report a case of a young femal...