Salivary glands
Normal Anatomy
IgG4 related dacryoadenitis and sialoadenitis (Mikulicz disease / syndrome)

Author: Sally Tanakchi, M.B.Ch.B. (see Authors page)
Editor: F. Zahra Aly, M.D., Ph.D.

Revised: 29 October 2015, last major update October 2015

Copyright: (c) 2002-2015, PathologyOutlines.com, Inc.

PubMed Search: IgG4 related dacryoadenitis and sialoadenitis
Cite this page: IgG4 related dacryoadenitis and sialoadenitis (Mikulicz disease / syndrome). PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/salivaryglandsmikulicz.html. Accessed December 7th, 2016.
Definition / General
  • Presents as painless, bilateral enlargement of lacrimal and salivary glands with mild secretory dysfunction
  • Today, the terms "Mikulicz disease" and "Mikulicz syndrome" are viewed as ambiguous and outdated by some sources
  • IgG4 related disease is now recognized as systemic disorder, characterized by high serum IgG4, marked infiltration of IgG4 positive plasma cells and severe fibrosis
  • The diagnosis depends on histologic appearance, elevated ratio of IgG4 / IgG, variable elevated serum IgG4 levels
  • Submandibular gland biopsy is more useful than labial salivary gland biopsy
  • Corticosteroids and rituximab (by targeting peripheral CD20-positive plasma cells) appear to be effective for IgG4 related diseases
Case Reports
Gross Description
  • Solid, gray-white areas and occasional cysts
Micro Description
  • Lymphoplasmacytic infiltration with lymphoid follicles surrounding solid epithelial nests (epimyoepithelial islands)
  • Also scattered histiocytes and dendritic cells
  • Excess hyaline basement membrane material deposited between cells
  • Mild acinar destruction, lymphoepithelial lesions, monocytoid B cells
  • Fibrosis has a characteristic irregular whorled pattern, termed "storiform fibrosis"
  • Multiple germinal center formation in granular tissue
Micro Images
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Figure 2B

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Lacrimal gland

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IgG4 positive plasma cells

Differential Diagnosis
  • Sjogren syndrome: no association with IgG4, female predominance, presence of SS-A and SS-B antibodies, severe acinar destruction