Name of Syndrome:
Weight: N/A Cause of Syndrome - Medical History (if
Vital Signs: HR:N/A
Respiratory rate: N/A Autosomal Dominant inherited Disease
Temperature:N/A The cause of this disease seems to be a germline mutation of a
tumor suppressor gene known as STK11/Lkb1. This gene has
Mealnae variable penetrances that cause differentiated phenotypic
Chief manifestations in people with Peutz-Jeghers. This means that the
complaint symptoms of the disease are similar in nature but vary between
different cases of the disease.
Age/race/sex predilections and Systemic Orofacial Clinical Features
Mucocutaneous pigmentation and melanin spots
on the peribuccal mucosa and around the
• Hamartomatous polyps that from in the vermillion border of the lips. The macules are
G.I tract mainly in the small intestines usually small brown or dark blue spots with the
and stomach. appearance of freckles.
•Intense abdominal pain Localization in the oral mucosa is common in
•Unexplained intestinal bleeding in a patients with PJS but the spots can also appear
young patient. on the hands and feet.
The spots usually are present at a young age and
•Prolapse of tissue from the rectum
fade at puberty except for lesions on the mucosa
•Menstral irregularities in women
•Prococious puberty Radiographic Features of this Syndrome
•Gynecomastia in males
Endoscopic ultrasound is now being used
•Race- Cases have been presented in all
for early detection of the polyps within the
races, no one more dominant than the
•Sex-Male and Female cases about equal
•Age- 23 in men, 26 in women
Special considerations in Treatment of this patient? How is it Diagnosed?
How common is it? Is it a horse or zebra?
PJS is said to occur in 1 in every 25-30 thousands births worldwide
I would consider it a zebra because although it is mainly inherited it is an actual
mutation of a gene.
There are no real considerations of this disease that require
specialized dental treatment in patients with PJS. However, it is
important for us to understand the disease because the
symptoms of it manifest in and around the oral cavity. We as
health care providers cannot diagnose this but we can sure be
the ones to detect this disease and refer patients to their
physicians regarding our findings. Our findings of the mucosal
pigmentation as well as endoscopic findings of intestinal polyps
confirm the diagnosis of PJS.
Pictures of Disease or Syndrome
List Sources and References
Bhattacharya, Sayantan. "Full Text | Melaena with Peutz-Jeghers Syndrome: a Case
Report." Journal of Medical Case Reports. 8 Feb. 2010. Web. 07 Nov. 2010.
"Google Images."/Peutz-Jeghers syndrome Google. Web. 07 Nov. 2010.
Ibsen, Olga A. C., Joan Andersen. Phelan, and Olga A. C. Ibsen. Oral Pathology for the
Dental Hygienist. St. Louis, MO: Saunders/Elsevier, 2009. Print.
Mukherjee, Sandeep. "Peutz-Jeghers Syndrome: EMedicine Gastroenterology."
EMedicine - Medical Reference. 9 Apr. 2009. Web. 07 Nov. 2010.