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Peutz-Jeghers syndrome without mucocutaneous pigmentation: a case report.

Mozaffar M, Sobhiyeh MR, Hasani M, Fallah M - Gastroenterol Hepatol Bed Bench (2012)

Bottom Line: Peutz-Jeghers syndrome is a rare condition characterized by mucocutaneous pigmentation, polyposis and an increased cancer risk at a number of gastrointestinal and extra intestinal organs.Upper and lower gastrointestinal endoscopy revealed multiple polyps located in the deuodenum and colon.Histopathological evaluation of the polyps revealed hamartomatous polyps of Peutz-Jeghers syndrome.

View Article: PubMed Central - PubMed

Affiliation: Department of Surgery, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

ABSTRACT
Peutz-Jeghers syndrome is a rare condition characterized by mucocutaneous pigmentation, polyposis and an increased cancer risk at a number of gastrointestinal and extra intestinal organs. We present a patient with a history of gastrointestinal bleeding with no mucocutaneous pigmentation. Upper and lower gastrointestinal endoscopy revealed multiple polyps located in the deuodenum and colon. Histopathological evaluation of the polyps revealed hamartomatous polyps of Peutz-Jeghers syndrome.

No MeSH data available.


Related in: MedlinePlus

No pigmentation was seen in mucocutaneous area
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Figure 0003: No pigmentation was seen in mucocutaneous area

Mentions: PJS is a rare familial disorder, with an incidence of 1 in 12-30,000 live births (4) and occurs with an estimated frequency of 1/8300 to 1/280,000 individuals (5) and has a male to female ratio of 1:1. The average age at the time of diagnosis is 23 years in men, and 26 years in women (6). PJS presents with characteristic flat, pigmented, freckle-like cutaneous lesions mainly on the lower lip, perioral area, buccal mucosa, periorbital area and eyelids (4). These lesions are benign and not thought to have malignant potential (5). Our patient did not have any pigmentation in mucocutaneous area (Figure 3).


Peutz-Jeghers syndrome without mucocutaneous pigmentation: a case report.

Mozaffar M, Sobhiyeh MR, Hasani M, Fallah M - Gastroenterol Hepatol Bed Bench (2012)

No pigmentation was seen in mucocutaneous area
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4017480&req=5

Figure 0003: No pigmentation was seen in mucocutaneous area
Mentions: PJS is a rare familial disorder, with an incidence of 1 in 12-30,000 live births (4) and occurs with an estimated frequency of 1/8300 to 1/280,000 individuals (5) and has a male to female ratio of 1:1. The average age at the time of diagnosis is 23 years in men, and 26 years in women (6). PJS presents with characteristic flat, pigmented, freckle-like cutaneous lesions mainly on the lower lip, perioral area, buccal mucosa, periorbital area and eyelids (4). These lesions are benign and not thought to have malignant potential (5). Our patient did not have any pigmentation in mucocutaneous area (Figure 3).

Bottom Line: Peutz-Jeghers syndrome is a rare condition characterized by mucocutaneous pigmentation, polyposis and an increased cancer risk at a number of gastrointestinal and extra intestinal organs.Upper and lower gastrointestinal endoscopy revealed multiple polyps located in the deuodenum and colon.Histopathological evaluation of the polyps revealed hamartomatous polyps of Peutz-Jeghers syndrome.

View Article: PubMed Central - PubMed

Affiliation: Department of Surgery, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

ABSTRACT
Peutz-Jeghers syndrome is a rare condition characterized by mucocutaneous pigmentation, polyposis and an increased cancer risk at a number of gastrointestinal and extra intestinal organs. We present a patient with a history of gastrointestinal bleeding with no mucocutaneous pigmentation. Upper and lower gastrointestinal endoscopy revealed multiple polyps located in the deuodenum and colon. Histopathological evaluation of the polyps revealed hamartomatous polyps of Peutz-Jeghers syndrome.

No MeSH data available.


Related in: MedlinePlus