Korean J Ophthalmol.  2012 Jun;26(3):216-221. 10.3341/kjo.2012.26.3.216.

Unusual Involvement of IgG4-Related Sclerosing Disease in Lacrimal and Submandibular Glands and Extraocular Muscles

• Shin YU
• Oh YH
• Lee YJ

• KMID: 1376129
• DOI: http://doi.org/10.3341/kjo.2012.26.3.216

Abstract

Chronic sclerosing sialadenitis, also known as Kuttner tumor, is a chronic inflammatory disease of the salivary glands that is reported in a few cases in medical literature. Recent reports suggest that certain aspects of sclerosing diseases, including chronic sclerosing sialadenitis or dacryoadenitis, should be classified under immunoglobulin G4 (IgG4)-related sclerosing disease based on immunohistochemical studies. This study reports an unusual case of IgG4-related sclerosing disease appearing simultaneously in the lacrimal glands, submandibular glands, and extraocular muscles. A 56-year-old male presented with complaints of bilateral eyelid swelling and proptosis that began two years ago. Computed tomography confirmed that bilateral submandibular enlargements also existed five years ago in the subject. Orbital computed tomography and magnetic resonance imaging revealed bilateral lacrimal gland enlargement and thickening of extraocular muscles. Typical findings of chronic sclerosing dacryoadenitis were revealed upon pathologic exam of the right lacrimal gland. Immunostaining revealed numerous IgG4-positive plasma cells. Through these clinical features, we make a diagnosis of IgG4-relataed sclerosing disease in the subject.

Keyword

Extraocular muscles; IgG4-related sclerosing disease; Kuttner tumor; Lacrimal apparatus

MeSH Terms

Biopsy, Fine-Needle
Diagnosis, Differential
Facial Muscles/*immunology/pathology/radiography
Follow-Up Studies
Humans
Immunoglobulin G/*immunology/metabolism
Immunohistochemistry
Lacrimal Apparatus/*immunology/metabolism/pathology
Lacrimal Duct Obstruction/complications/diagnosis/*immunology
Magnetic Resonance Imaging
Male
Middle Aged
Sclerosis
Submandibular Gland/*immunology/pathology/radiography
Submandibular Gland Diseases/complications/diagnosis/*immunology
Tomography, X-Ray Computed

Figure

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  Fig. 1 Complaint from a 56-year-old male of bilateral eyelid swelling and proptosis that started 2 years ago. Symptoms were more promi nent in the right eye. (A) Frontal view and (B) inferior view.

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  Fig. 2 (A) Both submandibular enlargements (arrows) existed 5 years ago. Currently there have been no definite changes of size. (B) Incisional biopsy of right lacrimal mass was performed. Enlargement of lacrimal gland was found (arrow).

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  Fig. 3 (A) Axial view of orbital computed tomography (CT). Arrows indicate bilateral lacrimal gland enlargement. (B) Axial view of orbital magnetic resonance imaging (MRI) also shows bilateral lacrimal gland enlargement (arrows). (C) Coronal view of orbital CT demonstrates the enlargement of bilateral lateral recti (arrows) and right inferior rectus. (D) Coronal view of orbital MRI. Dashed arrow indicates the hypertrophy of right inferior rectus.

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  Fig. 4 (A,B) Light microscopic examination of tissue stained with H&E. (A) Lacrimal gland reveals dense lymphocyte hyperplasia and lymphoid follicles (×2). (B) Atrophic lacrimal ducts and periductal sclerosis are shown (×40). (C,D) Tissue with immunohistochemical stains. The hyperplastic lymphoid tissues are composed of polyclonal lymphoid cells, positive for CD 3 (C, ×40) and positive for CD 20 (D, ×2).

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  Fig. 5 (A,B) Immunostaining for immunoglobulin G4 (IgG4). Abundant IgG4-positive plasma cells (brown color) are demonstrated. Prevalence shows that Kuttner tumor is a kind of IgG4-related sclerosing disease (A, ×100; B, ×200).

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