Orbit, 2014; 33(3): 217–219 ! Informa Healthcare USA, Inc. ISSN: 0167-6830 print / 1744-5108 online DOI: 10.3109/01676830.2014.884147
C ASE REPORT
IgG4-Related Lacrimal Sac Diverticulitis Satoru Kase1,2, Yasuo Suzuki1, Toshiya Shinohara3, and Manabu Kase1 1
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2
Department of Ophthalmology, Teine Keijinkai Hospital, Maeda 1-12, Teine-ku, Sapporo, Japan, Department of Ophthalmology, Hokkaido University, Hokkaido, Japan, and 3Department of Surgical Pathology, Teine Keijinkai Hospital
ABSTRACT IgG4-related disease is characterized by elevated serum IgG4 and tissue infiltration by IgG4-positive plasma cells. We herein report a case of lacrimal sac diverticulitis with marked IgG4-positive plasma cell infiltration. An 89-year-old woman presenting with right lower eyelid mass. Imaging modalities demonstrated a cystic orbital mass located beneath the globe and adjacent to enlarged lacrimal sac. Serological tests showed high IgG4 and normal IgG levels, measuring 242 and 1603 mg/dl, respectively. The orbital mass was surgically excised. Histologically, the excised tissue demonstrated marked inflammation with fibrosis surrounded by mononuclear epithelial cells. A variety of IgG and IgG4-positive plasma cells infiltrated the stroma. This patient was diagnosed as an IgG4-related lacrimal sac diverticulitis, based on current diagnostic criteria of IgG4-related disease. It is likely that IgG4-related inflammation occurs in a lacrimal sac diverticulum, which should be considered a differential diagnosis in inferior orbital tumors. Keywords: IgG4, inflammation, lacrimal sac diverticulitis
INTRODUCTION
December 2012. Her corrected visual acuities were mildly impaired due to senile cataract without abnormal fundus findings. A painless subcutaneous mass lesion with a smooth surface was palpable in the right eyelid (Figures 1A, B). Computed tomography demonstrated orbital masses located beneath the eye globe and adjacent to enlarged lacrimal sac (Figures 1C, D). The tumor showed cystic changes with heterogeneous isointensity and low-intensity by T1 and T2-weighted magnetic resonance imaging, respectively (Figures 1E, F). Systemic survey verified no other tumor except right orbit. Serological tests showed high IgG4 and normal IgG levels, measuring 242 mg/dl (normal: less than 105 mg/dl) and 1603 mg/dl (normal: less than 1700 mg/dl), respectively. One year after an initial presentation, the orbital tumor was excised following a subciliary cutaneous incision. The cystic tumor was adjacent to the lacrimal sac through the valve, containing myxoid fluid. Finally, the valve was closed by suturing. Histologically, the excised tissue demonstrated marked inflammation with fibrosis
IgG4-related disease is characterized by elevated serum IgG4 and tissue infiltration by IgG4-positive plasma cells1. IgG4-related ophthalmic disease includes not only dacryoadenitis2,3, but also other orbital tissue involvement such as the infraorbital nerve4 and lacrimal sac5. We herein report a case of the lacrimal sac diverticulum with marked IgG4positive plasma cell infiltration.
CASE REPORT An 89-year-old female complained of right lower eyelid swelling. She had a medical about 2 years ago history of bronchial asthma and diabetes mellitus without a history of acute dacryocystitis, ophthalmic surgery or trauma. She visited an eye clinic for evaluation of diabetic ocular complications. At this appointment, an orbital tumor was diagnosed, and she was subsequently referred to our hospital in
Received 17 July 2013; Revised 8 January 2014; Accepted 13 January 2014; Published online 24 February 2014 Correspondence: Satoru Kase, Department of Ophthalmology, Teine Keijinkai Hospital, Maeda 1-12, Teine-ku, Sapporo 006-0811, Japan. Tel: +81 11 681 8111. Fax: + 81 11 685 2998. E-mail: [email protected]
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218 S. Kase et al.
FIGURE 1. Facial photograph (A, B), and computed tomography (CT) (C, D) and magnetic resonance imaging (MRI) (E, F) at an initial presentation. The patient presents with right lower eyelid swelling (A, B). CT demonstrates an orbital mass, located beneath the eye globe (C, D). The tumor is adjacent to the enlarged lacrimal sac (E, F; arrow). The tumor contains cystic space showing heterogeneous iso-intensity and low-intensity by T1 and T2-weighted MRI, respectively.
FIGURE 2. Hematoxylin & eosin staining (A, B) and immunohistochemistry of IgG (C) and IgG4 (D) in the excised lacrimal fistula. Histologically, the excised tissue reveals marked inflammatory cell infiltration with fibrosis surrounded by mononuclear epithelial cells (A). At a high magnification, inflammatory cells are made up of plasma cells and lymphocytes where eosinophils are intermingled (B). A variety of IgG (C), and IgG4 (D)-positive plasma cells infiltrate the stroma.
surrounded by mononuclear epithelial cells (Figure 2A). At a high magnification, inflammatory cells were made up of plasma cells and lymphocytes where eosinophils were intermingled (Figure 2B). A variety of IgG and IgG4-positive plasma cells
infiltrated the stroma (Figures 2C, D). The number of IgG4-positive plasma cells was more than 40 in a high power field. Immunohistochemically, IgG4/IgG ratio was 82.5%. The patient is well without any additional treatments. Orbit
IgG4-related Lacrimal Sac Diverticulitis
Orbit Downloaded from informahealthcare.com by University of Washington on 03/01/15 For personal use only.
DISCUSSION Differential diagnoses of the orbital lesion in this patient can be lacrimal fistula. An acquired lacrimal fistula occurs in an improperly and inadequately treated lacrimal sac abscess,6 which usually shows a cutaneous fistula, following a spontaneously or iatrogenically ruptured or drained lacrimal abscess. As shown in this case, the orbital tumor was noted during observations by an ophthalmologist. However, the patient had no medical history of dacryocystitis, without a cutaneous fistula, suggesting that the tumor developed by different acquired nature from lacrimal fistula. The case report is essentially a diverticulum arising from the lacrimal sac with inflammatory cell infiltration, which should be considered a differential diagnosis in inferior orbital tumors. The patient showed high serum IgG4 levels. Histologically, the tissue contained mononuclear epithelial cells with marked IgG4-positive plasma cell infiltration in the stroma. Therefore, this patient can be diagnosed as IgG4-related inflammation of the orbit, based on current diagnostic criteria of IgG4-related disease.1,3 Sinnreich et al. described that acquired diverticula may result from recurrent dacryocystitis, which causes a localized weakening of the lacrimal sac wall.7 Therefore, the diverticulum formation in this case may be associated
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with IgG4-related chronic inflammation in the lacrimal sac.
DECLARATION OF INTEREST The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
REFERENCES 1. Umehara H, Okazaki K, Masaki Y, et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol 2012;22:21–30. 2. Kase S, Yamamoto T, Ishijima K, et al. Spontaneous regression of IgG4-related dacryoadenitis. Mod Rheumatol 2013;23:1018–1021. 3. Kase S, Noda M, Ishijima K, et al. IgG4-related inflammation of the orbit simulating malignant lymphoma. Anticancer Res 2013;33:2779–2783. 4. Sogabe Y, Miyatani K, Goto R, et al. Pathological findings of infraorbital nerve enlargement in IgG4-related ophthalmic disease. Jpn J Ophthalmol 2012;56:511–514. 5. Batra R, Mudhar HS, Sandramouli S. A unique case of IgG4 sclerosing dacryocystitis. Ophthal Plast Reconstr Surg 2012; 28:e70–e72. 6. Barrett RV, Meyer DR. Acquired lacrimal sac fistula after incision and drainage for dacryocystitis: a multicenter study. Ophthal Plast Reconstr Surg 2009;25:455–457. 7. Sinnreich Z. Lacrimal diverticula. Orbit 1998;17:195–200.
C ASE REPORT
IgG4-Related Lacrimal Sac Diverticulitis Satoru Kase1,2, Yasuo Suzuki1, Toshiya Shinohara3, and Manabu Kase1 1
Orbit Downloaded from informahealthcare.com by University of Washington on 03/01/15 For personal use only.
2
Department of Ophthalmology, Teine Keijinkai Hospital, Maeda 1-12, Teine-ku, Sapporo, Japan, Department of Ophthalmology, Hokkaido University, Hokkaido, Japan, and 3Department of Surgical Pathology, Teine Keijinkai Hospital
ABSTRACT IgG4-related disease is characterized by elevated serum IgG4 and tissue infiltration by IgG4-positive plasma cells. We herein report a case of lacrimal sac diverticulitis with marked IgG4-positive plasma cell infiltration. An 89-year-old woman presenting with right lower eyelid mass. Imaging modalities demonstrated a cystic orbital mass located beneath the globe and adjacent to enlarged lacrimal sac. Serological tests showed high IgG4 and normal IgG levels, measuring 242 and 1603 mg/dl, respectively. The orbital mass was surgically excised. Histologically, the excised tissue demonstrated marked inflammation with fibrosis surrounded by mononuclear epithelial cells. A variety of IgG and IgG4-positive plasma cells infiltrated the stroma. This patient was diagnosed as an IgG4-related lacrimal sac diverticulitis, based on current diagnostic criteria of IgG4-related disease. It is likely that IgG4-related inflammation occurs in a lacrimal sac diverticulum, which should be considered a differential diagnosis in inferior orbital tumors. Keywords: IgG4, inflammation, lacrimal sac diverticulitis
INTRODUCTION
December 2012. Her corrected visual acuities were mildly impaired due to senile cataract without abnormal fundus findings. A painless subcutaneous mass lesion with a smooth surface was palpable in the right eyelid (Figures 1A, B). Computed tomography demonstrated orbital masses located beneath the eye globe and adjacent to enlarged lacrimal sac (Figures 1C, D). The tumor showed cystic changes with heterogeneous isointensity and low-intensity by T1 and T2-weighted magnetic resonance imaging, respectively (Figures 1E, F). Systemic survey verified no other tumor except right orbit. Serological tests showed high IgG4 and normal IgG levels, measuring 242 mg/dl (normal: less than 105 mg/dl) and 1603 mg/dl (normal: less than 1700 mg/dl), respectively. One year after an initial presentation, the orbital tumor was excised following a subciliary cutaneous incision. The cystic tumor was adjacent to the lacrimal sac through the valve, containing myxoid fluid. Finally, the valve was closed by suturing. Histologically, the excised tissue demonstrated marked inflammation with fibrosis
IgG4-related disease is characterized by elevated serum IgG4 and tissue infiltration by IgG4-positive plasma cells1. IgG4-related ophthalmic disease includes not only dacryoadenitis2,3, but also other orbital tissue involvement such as the infraorbital nerve4 and lacrimal sac5. We herein report a case of the lacrimal sac diverticulum with marked IgG4positive plasma cell infiltration.
CASE REPORT An 89-year-old female complained of right lower eyelid swelling. She had a medical about 2 years ago history of bronchial asthma and diabetes mellitus without a history of acute dacryocystitis, ophthalmic surgery or trauma. She visited an eye clinic for evaluation of diabetic ocular complications. At this appointment, an orbital tumor was diagnosed, and she was subsequently referred to our hospital in
Received 17 July 2013; Revised 8 January 2014; Accepted 13 January 2014; Published online 24 February 2014 Correspondence: Satoru Kase, Department of Ophthalmology, Teine Keijinkai Hospital, Maeda 1-12, Teine-ku, Sapporo 006-0811, Japan. Tel: +81 11 681 8111. Fax: + 81 11 685 2998. E-mail: [email protected]
217
Orbit Downloaded from informahealthcare.com by University of Washington on 03/01/15 For personal use only.
218 S. Kase et al.
FIGURE 1. Facial photograph (A, B), and computed tomography (CT) (C, D) and magnetic resonance imaging (MRI) (E, F) at an initial presentation. The patient presents with right lower eyelid swelling (A, B). CT demonstrates an orbital mass, located beneath the eye globe (C, D). The tumor is adjacent to the enlarged lacrimal sac (E, F; arrow). The tumor contains cystic space showing heterogeneous iso-intensity and low-intensity by T1 and T2-weighted MRI, respectively.
FIGURE 2. Hematoxylin & eosin staining (A, B) and immunohistochemistry of IgG (C) and IgG4 (D) in the excised lacrimal fistula. Histologically, the excised tissue reveals marked inflammatory cell infiltration with fibrosis surrounded by mononuclear epithelial cells (A). At a high magnification, inflammatory cells are made up of plasma cells and lymphocytes where eosinophils are intermingled (B). A variety of IgG (C), and IgG4 (D)-positive plasma cells infiltrate the stroma.
surrounded by mononuclear epithelial cells (Figure 2A). At a high magnification, inflammatory cells were made up of plasma cells and lymphocytes where eosinophils were intermingled (Figure 2B). A variety of IgG and IgG4-positive plasma cells
infiltrated the stroma (Figures 2C, D). The number of IgG4-positive plasma cells was more than 40 in a high power field. Immunohistochemically, IgG4/IgG ratio was 82.5%. The patient is well without any additional treatments. Orbit
IgG4-related Lacrimal Sac Diverticulitis
Orbit Downloaded from informahealthcare.com by University of Washington on 03/01/15 For personal use only.
DISCUSSION Differential diagnoses of the orbital lesion in this patient can be lacrimal fistula. An acquired lacrimal fistula occurs in an improperly and inadequately treated lacrimal sac abscess,6 which usually shows a cutaneous fistula, following a spontaneously or iatrogenically ruptured or drained lacrimal abscess. As shown in this case, the orbital tumor was noted during observations by an ophthalmologist. However, the patient had no medical history of dacryocystitis, without a cutaneous fistula, suggesting that the tumor developed by different acquired nature from lacrimal fistula. The case report is essentially a diverticulum arising from the lacrimal sac with inflammatory cell infiltration, which should be considered a differential diagnosis in inferior orbital tumors. The patient showed high serum IgG4 levels. Histologically, the tissue contained mononuclear epithelial cells with marked IgG4-positive plasma cell infiltration in the stroma. Therefore, this patient can be diagnosed as IgG4-related inflammation of the orbit, based on current diagnostic criteria of IgG4-related disease.1,3 Sinnreich et al. described that acquired diverticula may result from recurrent dacryocystitis, which causes a localized weakening of the lacrimal sac wall.7 Therefore, the diverticulum formation in this case may be associated
!
2014 Informa Healthcare USA, Inc.
219
with IgG4-related chronic inflammation in the lacrimal sac.
DECLARATION OF INTEREST The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
REFERENCES 1. Umehara H, Okazaki K, Masaki Y, et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol 2012;22:21–30. 2. Kase S, Yamamoto T, Ishijima K, et al. Spontaneous regression of IgG4-related dacryoadenitis. Mod Rheumatol 2013;23:1018–1021. 3. Kase S, Noda M, Ishijima K, et al. IgG4-related inflammation of the orbit simulating malignant lymphoma. Anticancer Res 2013;33:2779–2783. 4. Sogabe Y, Miyatani K, Goto R, et al. Pathological findings of infraorbital nerve enlargement in IgG4-related ophthalmic disease. Jpn J Ophthalmol 2012;56:511–514. 5. Batra R, Mudhar HS, Sandramouli S. A unique case of IgG4 sclerosing dacryocystitis. Ophthal Plast Reconstr Surg 2012; 28:e70–e72. 6. Barrett RV, Meyer DR. Acquired lacrimal sac fistula after incision and drainage for dacryocystitis: a multicenter study. Ophthal Plast Reconstr Surg 2009;25:455–457. 7. Sinnreich Z. Lacrimal diverticula. Orbit 1998;17:195–200.
