Intermittent facial swelling, dry eyes and xerostomia
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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.
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
- 1. Fujita A, Sakai O, Chapman MN, Sugimoto H. IgG4-related disease of the head and neck: CT and MR imaging manifestations. (2012) Radiographics : a review publication of the Radiological Society of North America, Inc. 32 (7): 1945-58. doi:10.1148/rg.327125032 - Pubmed
- 2. Rao D, Natter P, Fernandes R, Wang ZB, Sandhu SJS. A Case Report of Mikulicz Syndrome. (2017) Journal of radiology case reports. 11 (7): 1-7. doi:10.3941/jrcr.v11i7.3147 - Pubmed