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Basal Cell Nevus Syndrome

Overview

It is an inherited disorder which means that it runs in a family.It is autosomal dominant condition in which if a single gene id defective then it passes on to the other generation as well as it has dominant nature.In this diseases there is abnormalities in the skin, nervous tissues, eyes, endocrine disorder and bones.There is unusual facial appearance on the patient who is suffering from the disease.The patients who is being affected by a disease has 10% chances to develop the skin cancer. UV radiation are the primary factor for causing these cancers.There are different levels of symptoms for each individual who are affected by these diseases.


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Causes

The cause lies behind the changed genome of individual due to the UV rays.The mutation lie in the PATCH gene found on the chromosome 9Th on its short arm.It is autosomal dominant inherited condition.It is a rare condition.


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Signs & Symptoms

    * Patients with this syndrome have basal cell carcinoma

 

    * Ribs and vertebrae anomalies

 

    * Intracranial tension

 

    * Frontal bossing and temporpariental bossing

 

    * Heloteroism

 

    * Prognathism(promineent chin)

 

    * Saddle shaped nose

 

    * Pal mar pits

 

    * Ondotogenic cysts are  like the cysts on the posterior part of the mandible.

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Prevention

    * Genetic counselling

 

    * Reducing exposure to the sunlight

 

    * Radiation therapy cannot be used in skin cancers and they are advised for  the other symptoms involving the other systems like CNS, CVS, etc


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Treatments
Genetic counselling Psychotherapy

    * Genetic counselling

 

    * Severity of the basal cell carcinoma determine the prognosis of the disease.

 

    * Enucleation of the odontogenic cysts..

 

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