Orbital Inflammation: A Rare Association of Common Variable Immunodeficiency

Mehta, Purnima; Chickadasarahally, Swaroop; Ahluwalia, Harpreet

doi:10.3109/01676830.2010.539769

Cited by 5 publications

(1 citation statement)

References 6 publications

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“…There are several reports of ocular manifestations of granulomatous CVID, including granulomatous uveitis [18,19,20,21]. Autoimmune and non-granulomatous chronic inflammatory complications of CVID include uveitis, diffuse placoid chorioretinopathy, bird-shot chorioretinopathy, bilateral corneal ulcerations and orbital inflammations [22,23,24,25,26,27,28,29,30]. Starr et al [31 ]noted an unusual pattern of retinitis pigmentosa and CVID in one family.…”

Section: Discussionmentioning

confidence: 99%

Metachronous Diffuse Large B-Cell Lymphoma and Kaposi Sarcoma of the Right Eyelid and Lacrimal Gland in a Patient with Granulomatous Common Variable Immunodeficiency

et al. 2016

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Purpose: To describe the ophthalmic and histopathological features of a female with granulomatous common variable immunodeficiency (CVID) who presented with upper-lid swelling. Procedures: The patient underwent a biopsy of the right upper lid/palpebral lacrimal gland with imaging showing a left-sided nasopharyngeal mass, multiple lymph nodes within the mediastinum, bilateral lung nodules and a peritoneal nodule in the right iliac fossa. The right upper-lid swelling progressed and was subject to a second biopsy. Results: The first right upper-lid biopsy revealed a diffuse large B-cell lymphoma (DLBCL), confirmed with clonal IgH gene rearrangement with PCR. The nasopharyngeal mass and lymph nodes were suspected clinically to be DLBCL. However, a biopsy of the nasopharyngeal mass showed Kaposi sarcoma (KS). The second biopsy of the right upper lid/palpebral lacrimal gland revealed KS with no evidence of DLBCL. Conclusion: This is the first documentation of periocular/orbital metachronous DLBCL and KS in a patient with granulomatous CVID. We discuss the role of fluctuating immunity in CVID to explain the spontaneous regression of the DLBCL and the varying clinical picture.

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Section: Discussionmentioning

confidence: 99%

Metachronous Diffuse Large B-Cell Lymphoma and Kaposi Sarcoma of the Right Eyelid and Lacrimal Gland in a Patient with Granulomatous Common Variable Immunodeficiency

et al. 2016

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Ocular Manifestations of Allergic and Immunologic Diseases

Christensen

McDonnell

Singh

2017

The Eye in Pediatric Systemic Disease

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Ocular Involvement in Primary Immunodeficiency Diseases

et al. 2013

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Primary immunodeficiency diseases (PID) are a group of inherited disorders characterized by recurrent infections, and in many cases autoimmunity and malignancies. A number of PID patients suffer from a variety of ocular manifestations. Although these associated ocular features are not common, awareness combined with better understanding of the contributing mechanisms will allow prompt diagnosis and specific treatment, leading to reduction or prevention of serious visual morbidities.

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Orbital Inflammation: A Rare Association of Common Variable Immunodeficiency

Cited by 5 publications

References 6 publications

Metachronous Diffuse Large B-Cell Lymphoma and Kaposi Sarcoma of the Right Eyelid and Lacrimal Gland in a Patient with Granulomatous Common Variable Immunodeficiency

Metachronous Diffuse Large B-Cell Lymphoma and Kaposi Sarcoma of the Right Eyelid and Lacrimal Gland in a Patient with Granulomatous Common Variable Immunodeficiency

Ocular Manifestations of Allergic and Immunologic Diseases

Ocular Involvement in Primary Immunodeficiency Diseases

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