Peutz Jeghers syndrome (PJS) is an autosomal dominant genetic condition characterized by multiple benign polyps called hamartomas on the mucous lining in the gastrointestinal system. These polyps occur most often in the small intestine but also occur in the stomach and large intestine. Affected individuals also have dark skin discoloration, especially around the eyes, nostrils, mucous membranes of the mouth, perianal area and inside the mouth. Affected individuals have an increased risk for intestinal and other cancers.

Hutchinson weber putz syndrome is caused by the autosomal dominant disorder which results in the numerous noncancerous benign polyps in the digestive system. These non-cancerous growths are called hamartomatous polyps. There is an increased risk of cancer associated with this disease.

• This condition is commonly seen in children.
• They tend to develop small dark-coloured spots on the mouth orifice and lips.
• The lesions can also be seen in the oral mucosa, eyes, nostrils and around the anus.
• This condition usually gets fades in adulthood.
• The symptoms of the disease include the health problems like repeated bowel obstructions, chronic bleeding and Abdominal pain.
• Sometimes the children can have a high risk of developing gastrointestinal cancer as well. They can also develop cancers in the pancreas, cervix, anus, and breast tissue.
• The patients are presented with restlessness and severe pain due to the discomfort caused by the polyps.
• The polyps tend to have a high blood supply which will increase the chances of bleeding even on simple touch or probing.
• This condition also can lead to nutritional deficiency due to poor food intake.
• The symptoms get severe in adolescents due to the hormonal changes in the body.
• It may usually fade as the patient reaches early adulthood.
• Proper screening and symptomatic removal of the polyps is the only recommended solution for the disease.

Conditions
Hamartomous polyps in oral cavity and nostrils
Drugs
Constant surveilance and test,Symptomatic treatment
Symptoms
Characteristic dark-colored spots,Development of hamartomatous polyps,Small bowel obstruction (blockage),Gastrointestinal bleeding,Anemia,Stomach pain,Intussusception of the small bowel

Hutchinson weber putz syndrome is caused due to the genetic mutation caused in the SK11 gene. This condition presents as the number of non-cancerous growths and polyps in the gastrointestinal tract and other mucosae of the body. These polyps are usually referred to as hamartomatous polyps.

• The patients are usually children with numerous and discolored spots in the oral and anal mucosa.
• They also present with painful polyp growth in the nostrils, lips, stomach, and intestine.
• This reduces their nutrition intake, which leads to severe issues in the future.
• Hutchinson Weber Peutz (HWPS) is caused by a mutation in one copy of the STK11/LKB1 gene.
• Everyone has two copies of the STK11/LKB1 gene. Sometimes the mutation is seen in both copies.
• This causes numerous growth in the gastrointestinal tracts.
• This disease is an autosomal dominant pattern.
• It means that this disease can be transmitted to all children if any of the parents possess the disease.
• Sometimes, some children have the first generation mutation of these particular genes.
• Preimplantation genetic analysis of the offspring shows the presence of the disease.
• It can also be caused due to the effects of other genes. These genes are yet to be discovered.
• There are also idiopathic conditions of these diseases seen across the world.
• The reason for such a condition is not known to date. It is a relatively low incidence of disease but has significance as there is a high risk of cancer in these patients.
• Timely endoscopy and colonoscopy, and other screening processes will reduce the severity of the disease.

Conditions
Hamartomous polyps in oral cavity and nostrils
Drugs
Constant surveilance and test,Symptomatic treatment
Symptoms
Characteristic dark-colored spots,Development of hamartomatous polyps,Small bowel obstruction (blockage),Gastrointestinal bleeding,Anemia,Stomach pain,Intussusception of the small bowel

Hutchinson Weber Peutz Syndrome is an auto-immune disorder caused due to a genetic mutation. The change caused in the structure of the STK11 gene is an important reason for this condition.

Treatments-

• There is no specific treatment or cure for this disease.
• Patients must go out for prolonged surveillance when they suffer from this disease.
• Colonoscopy and endoscopy should be done at regular intervals.
• If any of the growths are seen in the stomach or bowels, they should be immediately removed as they have a severe risk of cancer.
• They may also cause intestinal blockage, affecting the working of the digestive system.
• Painful polyps should also be removed if they are seen in the nasal or oral mucosa.
• The patients should also be given nutritional supplements as they may not be able to achieve the same via a regular diet.
• Women should undergo routine mammograms and pap smear tests at least once in 6 months to deduct cancers.
• Similarly, men are advised to take annual testicular CT and prostrate checks.
• Since the disease tends to have an increased impact during the adolescent period, that period should be under serious surveillance.
• The patients should also avoid a diet that may be irritative to the bowel and practice a healthy lifestyle.

Conditions
Hamartomous polyps in oral cavity and nostrils
Drugs
Constant surveilance and test,Symptomatic treatment
Symptoms
Characteristic dark-colored spots,Development of hamartomatous polyps,Small bowel obstruction (blockage),Gastrointestinal bleeding,Anemia,Stomach pain,Intussusception of the small bowel

Hutchinson Weber Peutz syndrome/Peutz-Jeghers syndrome is a genetic condition that causes non-cancerous growths to develop in the digestive system.

Risk factors of Hutchinson Weber Peutz syndrome include,

• It is an autosomal dominant genetic condition caused by mutations in the STK11/LKB1 gene.
• The condition can be passed from generation to generation in a family.
• When only a single copy of an abnormal gene is necessary for the appearance of the disease, dominant genetic disorders occur.
• The abnormal gene can be inherited from either parent, or can be the result of a new mutation in the affected individual.
• There are 50% chances of passing the abnormal gene from an affected parent to an offspring for each pregnancy.
• The risk of developing Hutchinson Weber Peutz syndrome is the same for males and females.
• Individuals with this genetic condition have an increased risk of developing cancer and polyps.
• It is possible that there are other genes that could cause PJS that have not yet been discovered.

Conditions
Hamartomous polyps in oral cavity and nostrils
Drugs
Constant surveilance and test,Symptomatic treatment
Symptoms
Characteristic dark-colored spots,Development of hamartomatous polyps,Small bowel obstruction (blockage),Gastrointestinal bleeding,Anemia,Stomach pain,Intussusception of the small bowel

Currently, there is no cure for Hutchinson Weber Peutz syndrome.

• Patients may undergo lifelong surveillance of organs to monitor for cancer and prevent secondary problems from the polyps.
• As per studies patients may not need emergency small intestinal surgery if they are under close surveillance and do not develop cancer. This depends on polyps being removed before they are large enough to develop into cancer.
• Polyps can be easily removed during a colonoscopy.
• These Polyps present in the stomach and duodenum can be biopsied and removed if needed.
• Polyps present in the small bowel further downstream can be seen through a balloon or double balloon enteroscopy and can be removed.
• However, if the polyps cannot be reached, and there are symptoms of a greater issue, then surgery may be performed to remove it.
• Generally, the polyps can be removed one-by-one without losing any of the bowel.

Conditions
Hamartomous polyps in oral cavity and nostrils
Drugs
Constant surveilance and test,Symptomatic treatment
Symptoms
Characteristic dark-colored spots,Development of hamartomatous polyps,Small bowel obstruction (blockage),Gastrointestinal bleeding,Anemia,Stomach pain,Intussusception of the small bowel