A Case of IgG4-Related Sclerosing Disease Involving the Eyelid in an Idiopathic Sclerosing Myositis Patient

Je Hwan Yoon; Jee Woong Jung; Mi Jung Chi

doi:10.3341/jkos.2013.54.1.160

Journal List > J Korean Ophthalmol Soc > v.54(1) > 1009515

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Yoon, Jung, and Chi: A Case of IgG4-Related Sclerosing Disease Involving the Eyelid in an Idiopathic Sclerosing Myositis Patient

Original Article

J Korean Ophthalmol Soc 2013;54(1):160-164.

Published online: 25 September 2013

DOI: https://doi.org/10.3341/jkos.2013.54.1.160

A Case of IgG4-Related Sclerosing Disease Involving the Eyelid in an Idiopathic Sclerosing Myositis Patient

Je Hwan Yoon, MD, Jee Woong Jung, MD, Mi Jung Chi, MD, PhD

Department of Ophthalmology, Gachon University Gil Medical Center, Incheon, Korea

Address reprint requests to Mi Jung Chi, MD, PhD Department of Ophthalmology, Gachon University Gil Medical Center #21 Namdong-daero774beon-gil, Namdong-gu, Incheon 405-760, Korea Tel: 82-32-460-3364, Fax: 82-32-460-3358, E-mail: cmj@gilhospital.com

Received 20 December 2011 Accepted 16 March 2012

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Purpose

To report a case of IgG4-related sclerosing disease involving the eyelid in an idiopathic sclerosing myositis patient.

Case summary

A 51-year-old woman presented with swelling, redness, and tenderness of the left lower eyelid of 1 month duration had taken an immunosuppressant for idiopathic sclerosing myositis. Eye movements showed limitation all directions but there was no exophthalmos. A palpable mass was noted in the left lower eyelid. The left extraocular muscles were hypertrophied but the lacrimal gland was normal on orbital magnetic resonance imaging. IgG4-related sclerosing disease was confirmed by immunostained biopsy from the left lower eyelid, showing infiltration of IgG4-positive lymphoplasmacytic cells. The patient was given oral steroids and an immunosuppressant and the symptoms did not recur for at least 7 months.

Conclusions

IgG4-related sclerosing disease involving ocular adnexa usually consists of bilateral lacrimal gland involvement. Additionally, the orbital soft tissue involvement without dacryoadenitis is rare. The authors of the present study report a case of IgG4-related sclerosing disease involving the left lower eyelid in an idiopathic sclerosing myositis patient and should be considered in the differential diagnosis of eyelid masses.

Keywords: Eyelid, Idiopathic sclerosing myositis, IgG4-related sclerosing disease, Lacrimal gland

References

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Figure 1.

(A, B) An erythematous swelling was observed in the left lower eyelid.

Figure 2.

(A, B) Orbital T1-weighted MRIs. The images show swelling of the left medial rectus muscle and superior rectus muscle with tendon.

Figure 3.

(A) Biopsied specimen from left lower eyelid shows atrophied myofibers, prominent lymphoplasmacytic infiltration (H & E, ×1000) (black arrow), (B) IgG4-positive plasma cells count up to 19 per high power field (Anti-IgG4 Ab, ×400) (black arrow).

Figure 4.

The erythematous swelling of the left lower eyelid had improved and the symptom hadn't recurred after 7 months after treatment.

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