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Case Reports
. 2020 Nov 19:37:252.
doi: 10.11604/pamj.2020.37.252.26835. eCollection 2020.

A case report of Mikulicz's disease

Dang Luu Vu  1   2 , Tra My Thieu-Thi  2 , Minh Thong Pham  1   2 , My Le-Thi  1 , Huu An Nguyen  1 , Minh Duc Nguyen  2   3   4
Affiliations
Case Reports

A case report of Mikulicz's disease

Dang Luu Vu et al. Pan Afr Med J. .

Abstract

Mikulicz's disease is a unique condition involving the enlargement of the lacrimal and salivary glands, similar to that observed in Sjogren's syndrome; however, Mikulicz's disease is clinically characterized by infrequent autoimmune reactions and responsiveness to glucocorticoid treatment. The ultrasound features of the lacrimal and salivary glands in patients with IgG4-Mikulicz's disease were characterized by multiple hypoechoic areas of varying sizes within the enlarged glands. IgG4 serum level was also elevated, in contrast to the detection of normal levels in Sjogren's syndrome. In this article, we intended to illustrate a case of Mikulicz's disease with clinical and imaging features.

Keywords: Mikulicz’s disease; case report; lacrimal gland; salivary gland; swelling.

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Conflict of interest statement

The authors declare no competing interests.

Figures

Figure 1
Figure 1
ultrasound of the lacrimal glands before treatment: the left (A) and right (B) lacrimal glands were enlarged (left: 30 x 14 mm and right: 26 x 14 mm) before treatment, with multiple hypoechoic areas; C, D) both glands displayed hypervascularity
Figure 2
Figure 2
ultrasound of the lacrimal glands after treatment: left (A) and right (B) lacrimal glands decreased in size (left: 9 x 4 mm and right: 8 x 5 mm), and the vascularity and hypoechoic areas disappeared in response to corticosteroid therapy
Figure 3
Figure 3
ultrasound of the submandibular glands: A, B) the submandibular glands before treatment showed bilateral enlargement with multiple hypoechoic, hypervascular areas; C) the submandibular glands after treatment showed a decrease in the size and vascularity of the glands, in addition to the disappearance of hypoechoic areas
Figure 4
Figure 4
MRI showed enlarged lacrimal glands before treatment: A, B) fat-suppressed T2-weighted images demonstrated the hypointense bilateral swelling of the lacrimal glands; C) the lacrimal glands were isointense to skeletal muscle on T1-weighted images; D) T1-weighted images post-contrast showed that the lacrimal glands are remarkably and homogeneously enhanced
Figure 5
Figure 5
facial MRI before treatment: A) fat-suppressed T2-weighted images showed that the submandibular glands were diffusely enlarged and hypointense; B) T1-weighted images demonstrated the low signal intensity of submandibular glands; C) T1-weighted images with contrast showed the remarkably homogeneous enhancement of these glands
Figure 6
Figure 6
microscopic images of the lacrimal gland visualized with hematoxylin and eosin stain (A-C) and CD138 antibody stain (D); A) lymphoplasmacytic infiltration and fibrosis; B) fibrosclerosis was found in the gland interstitium; C) lacrimal ducts were observed with collagenous sheaths; D) CD38 immunoreactivity was observed, confirming plasma cell infiltration
Figure 7
Figure 7
microscopic images of lacrimal gland with anti-IgG antibody stain x100 (A); anti-IgG4 antibody stain x100 (B) and anti-IgG4 antibody stain x400 (C); A, B) representative section of the lacrimal gland showed infiltration of IgG and IgG4 cells; C) markedly increased IgG4-positive plasma cells (the number of IgG4+ cells >50/ high-power field)
See this image and copyright information in PMC

References

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