Mikulicz syndrome

Case contributed by Omar Wishyar Jamal

Presentation

Intermittent facial swelling, dry eyes and xerostomia

Patient Data

Age: 55 years
Gender: Female
CT

Bilateral parotid gland enlargement with hyperdense foci (calcifications) bilaterally associated with submandibular and lacrimal glands enlargement.

Several lymph nodes were seen bilaterally, of normal shape and preserved hilum.

Mild mucosal thickening of the maxillary sinuses also noted.

Case Discussion

Bilateral parotid gland enlargement has a wide range of differential diagnoses, however for this case Mikulicz disease is favored due to:

  • involvement of two or more salivary glands and lacrimal glands
  • history of intermittent facial swelling, dry eyes and xerostomia
  • ANA was positive with negative anti-Ro
  • IgG4 levels were elevated
  • histopathological examination revealed lymphocytes with a predominance of mononuclear cells
  • good response to corticosteroids

It is important to suggest Mikulicz syndrome because studies show that affected patients are 43% more likely to get lymphoma than the general population.

Also important to screen for other IgG4 diseases in these cases

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Case information

rID: 64328
Published: 3rd Dec 2018
Last edited: 14th Aug 2019
System: Head & Neck
Inclusion in quiz mode: Included
Institution: Rizgary Teaching Hospital

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