Friday, July 11, 2008
Peutz-Jeghers syndrome
I have never seen this till my practice for the MRCP exam. Well, there are some cases where you will never see before in your whole life except from atlas or websites and it appears for the first time during your MRCP examination. Don't get drawn back. Always stay calm and show the examiner that it is an easy case and talk confidently although you know nuts about it. Remember that the examiner cannot ask you much in the PACES exam because time is short and remember that they are physicians and not dermatologists !
There are small pigmented macules on the lips and buccal mucosa, suggestive of Peutz-Jeghers syndrome. I would like to ask the patient for any abdominal pain or previous history of GI bleed. I would also like to ask for family history of similar problem as it is autosomal dominant.
Peutz-Jeghers is associated with intestinal polyposis which are hamartomas and there is increased incidence of breast, ovarian and pancreatic malignancy.
Other differential diagnosis for this type of hyperpigmentation would include Addison's disease.
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